Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Entry version 205 (07 Apr 2021)
Sequence version 3 (10 Feb 2009)
Previous versions | rss
Add a publicationFeedback
Protein

Peroxisomal bifunctional enzyme

Gene

EHHADH

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score:

Annotation score:5 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
-Experimental evidence at protein leveli <p>This indicates the type of evidence that supports the existence of the protein. Note that the 'protein existence' evidence does not give information on the accuracy or correctness of the sequence(s) displayed.<p><a href='/help/protein_existence' target='_top'>More...</a></p>

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

Peroxisomal trifunctional enzyme possessing 2-enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase, and delta 3, delta 2-enoyl-CoA isomerase activities. Catalyzes two of the four reactions of the long straight chain fatty acids peroxisomal beta-oxidation pathway. Optimal isomerase for 2,5 double bonds into 3,5 form isomerization in a range of enoyl-CoA species (Probable). Also able to isomerize both 3-cis and 3-trans double bonds into the 2-trans form in a range of enoyl-CoA species (By similarity). With HSD17B4, catalyzes the hydration of trans-2-enoyl-CoA and the dehydrogenation of 3-hydroxyacyl-CoA, but with opposite chiral specificity (PubMed:15060085). Regulates the amount of medium-chain dicarboxylic fatty acids which are essential regulators of all fatty acid oxidation pathways (By similarity). Also involved in the degradation of long-chain dicarboxylic acids through peroxisomal beta-oxidation (PubMed:15060085).By similarity1 Publication1 Publication

Miscellaneous

Absent in patients suffering with peroxisomal disorders such as Zellweger syndrome, neonatal adrenoleukodystrophy and infantile Refsum disease.

<p>This subsection of the <a href="http://www.uniprot.org/help/function%5Fsection">Function</a> section describes the catalytic activity of an enzyme, i.e. a chemical reaction that the enzyme catalyzes.<p><a href='/help/catalytic_activity' target='_top'>More...</a></p>Catalytic activityi

<p>This subsection of the <a href="http://www.uniprot.org/help/function%5Fsection">Function</a> section describes regulatory mechanisms for enzymes, transporters or microbial transcription factors, and reports the components which regulate (by activation or inhibition) the reaction.<p><a href='/help/activity_regulation' target='_top'>More...</a></p>Activity regulationi

Enzyme activity enhanced by acetylation.1 Publication

<p>This subsection of the 'Function' section describes biophysical and chemical properties, such as maximal absorption, kinetic parameters, pH dependence, redox potentials and temperature dependence.<p><a href='/help/biophysicochemical_properties' target='_top'>More...</a></p>Kineticsi

  1. KM=0.3 µM for (3S)-hydroxyhexadecanedioyl-CoA1 Publication
  2. KM=10 µM for (3S)-hydroxyhexadecanoyl-CoA1 Publication

    <p>This subsection of the <a href="http://www.uniprot.org/help/function%5Fsection">'Function'</a> section describes the metabolic pathway(s) associated with a protein.<p><a href='/help/pathway' target='_top'>More...</a></p>Pathwayi: fatty acid beta-oxidation

    This protein is involved in the pathway fatty acid beta-oxidation, which is part of Lipid metabolism.1 Publication
    View all proteins of this organism that are known to be involved in the pathway fatty acid beta-oxidation and in Lipid metabolism.

    Sites

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the <a href="http://www.uniprot.org/help/function%5Fsection">Function</a> section describes the interaction between a single amino acid and another chemical entity. Priority is given to the annotation of physiological ligands.<p><a href='/help/binding' target='_top'>More...</a></p>Binding sitei101Substrate; via amide nitrogenBy similarity1
    <p>This subsection describes interesting single amino acid sites on the sequence that are not defined in any other subsection. This subsection can be displayed in different sections ('Function', 'PTM / Processing', 'Pathology and Biotech') according to its content.<p><a href='/help/site' target='_top'>More...</a></p>Sitei104Important for catalytic activityBy similarity1
    Sitei124Important for catalytic activityBy similarity1

    <p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

    GO - Biological processi

    <p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsi

    Molecular functionIsomerase, Lyase, Multifunctional enzyme, Oxidoreductase
    Biological processFatty acid metabolism, Lipid metabolism
    LigandNAD

    Enzyme and pathway databases

    BioCyc Collection of Pathway/Genome Databases

    More...
    BioCyci
    MetaCyc:HS03720-MONOMER

    Pathway Commons web resource for biological pathway data

    More...
    PathwayCommonsi
    Q08426

    Reactome - a knowledgebase of biological pathways and processes

    More...
    Reactomei
    R-HSA-390247, Beta-oxidation of very long chain fatty acids
    R-HSA-9033241, Peroxisomal protein import

    SABIO-RK: Biochemical Reaction Kinetics Database

    More...
    SABIO-RKi
    Q08426

    UniPathway: a resource for the exploration and annotation of metabolic pathways

    More...
    UniPathwayi
    UPA00659

    Chemistry databases

    SwissLipids knowledge resource for lipid biology

    More...
    SwissLipidsi
    SLP:000000543

    <p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

    <p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein.<p><a href='/help/protein_names' target='_top'>More...</a></p>Protein namesi
    Recommended name:
    Peroxisomal bifunctional enzymeCurated
    Short name:
    PBE
    Short name:
    PBFE
    Alternative name(s):
    L-bifunctional protein1 Publication
    Short name:
    LBP1 Publication
    Multifunctional enzyme 1
    Short name:
    MFE1
    Including the following 2 domains:
    Enoyl-CoA hydratase/3,2-trans-enoyl-CoA isomerase (EC:4.2.1.17, EC:5.3.3.8)
    3-hydroxyacyl-CoA dehydrogenase (EC:1.1.1.35)
    <p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section indicates the name(s) of the gene(s) that code for the protein sequence(s) described in the entry. Four distinct tokens exist: 'Name', 'Synonyms', 'Ordered locus names' and 'ORF names'.<p><a href='/help/gene_name' target='_top'>More...</a></p>Gene namesi
    Name:EHHADHImported
    Synonyms:ECHD
    <p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section provides information on the name(s) of the organism that is the source of the protein sequence.<p><a href='/help/organism-name' target='_top'>More...</a></p>OrganismiHomo sapiens (Human)
    <p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section shows the unique identifier assigned by the NCBI to the source organism of the protein. This is known as the 'taxonomic identifier' or 'taxid'.<p><a href='/help/taxonomic_identifier' target='_top'>More...</a></p>Taxonomic identifieri9606 [NCBI]
    <p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section contains the taxonomic hierarchical classification lineage of the source organism. It lists the nodes as they appear top-down in the taxonomic tree, with the more general grouping listed first.<p><a href='/help/taxonomic_lineage' target='_top'>More...</a></p>Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    <p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section is present for entries that are part of a <a href="http://www.uniprot.org/proteomes">proteome</a>, i.e. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.<p><a href='/help/proteomes_manual' target='_top'>More...</a></p>Proteomesi
    • UP000005640 <p>A UniProt <a href="http://www.uniprot.org/manual/proteomes%5Fmanual">proteome</a> can consist of several components.<br></br>The component name refers to the genomic component encoding a set of proteins.<p><a href='/help/proteome_component' target='_top'>More...</a></p> Componenti: Chromosome 3

    Organism-specific databases

    Human Gene Nomenclature Database

    More...
    HGNCi
    HGNC:3247, EHHADH

    Online Mendelian Inheritance in Man (OMIM)

    More...
    MIMi
    607037, gene

    neXtProt; the human protein knowledge platform

    More...
    neXtProti
    NX_Q08426

    Eukaryotic Pathogen, Vector and Host Database Resources

    More...
    VEuPathDBi
    HostDB:ENSG00000113790.10

    <p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

    Keywords - Cellular componenti

    Peroxisome

    <p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

    <p>This subsection of the 'Pathology and Biotech' section provides information on the disease(s) associated with genetic variations in a given protein. The information is extracted from the scientific literature and diseases that are also described in the <a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=omim">OMIM</a> database are represented with a <a href="http://www.uniprot.org/diseases">controlled vocabulary</a> in the following way:<p><a href='/help/involvement_in_disease' target='_top'>More...</a></p>Involvement in diseasei

    Fanconi renotubular syndrome 3 (FRTS3)1 Publication
    The disease is caused by variants affecting the gene represented in this entry.
    Disease descriptionA form of Fanconi renotubular syndrome, a disease due to a generalized dysfunction of the proximal kidney tubule resulting in decreased solute and water reabsorption. Patients have polydipsia and polyuria with phosphaturia, glycosuria and aminoaciduria. They may develop hypophosphatemic rickets or osteomalacia, acidosis and a tendency toward dehydration. Some eventually develop renal insufficiency. FRTS3 inheritance is autosomal dominant.
    Related information in OMIM
    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the 'Sequence' section describes natural variant(s) of the protein sequence.<p><a href='/help/variant' target='_top'>More...</a></p>Natural variantiVAR_0709493E → K in FRTS3; the mutant is mistargeted to mitochondria; results in impaired mitochondrial oxidative phosphorylation and defects in the transport of fluids across the epithelium of renal proximal tubular cells. 1 PublicationCorresponds to variant dbSNP:rs398124646EnsemblClinVar.1

    Mutagenesis

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the <a href="http://www.uniprot.org/manual/pathology%5Fand%5Fbiotech%5Fsection">'Pathology and Biotech'</a> section describes the effect of the experimental mutation of one or more amino acid(s) on the biological properties of the protein.<p><a href='/help/mutagen' target='_top'>More...</a></p>Mutagenesisi165K → Q: Greatly reduced acetylation and insensitive to treatment with TSA and NAM; when associated with Q-171; Q-346 and Q-584. 1 Publication1
    Mutagenesisi171K → Q: Greatly reduced acetylation and insensitive to treatment with TSA and NAM; when associated with Q-165; Q-346 and Q-584. 1 Publication1
    Mutagenesisi346K → Q: Greatly reduced acetylation and insensitive to treatment with TSA and NAM; when associated with Q-165; Q-171 and Q-584. 1 Publication1
    Mutagenesisi584K → Q: Greatly reduced acetylation and insensitive to treatment with TSA and NAM; when associated with Q-165; Q-171 and Q-346. 1 Publication1

    Keywords - Diseasei

    Disease variant

    Organism-specific databases

    DisGeNET

    More...
    DisGeNETi
    1962

    MalaCards human disease database

    More...
    MalaCardsi
    EHHADH
    MIMi615605, phenotype

    Open Targets

    More...
    OpenTargetsi
    ENSG00000113790

    Orphanet; a database dedicated to information on rare diseases and orphan drugs

    More...
    Orphaneti
    300, Bifunctional enzyme deficiency
    3337, Primary Fanconi renotubular syndrome

    The Pharmacogenetics and Pharmacogenomics Knowledge Base

    More...
    PharmGKBi
    PA27682

    Miscellaneous databases

    Pharos NIH Druggable Genome Knowledgebase

    More...
    Pharosi
    Q08426, Tbio

    Chemistry databases

    Drug and drug target database

    More...
    DrugBanki
    DB00157, NADH

    Genetic variation databases

    BioMuta curated single-nucleotide variation and disease association database

    More...
    BioMutai
    EHHADH

    Domain mapping of disease mutations (DMDM)

    More...
    DMDMi
    223590229

    <p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

    Molecule processing

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the 'PTM / Processing' section describes the extent of a polypeptide chain in the mature protein following processing or proteolytic cleavage.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_00001092471 – 723Peroxisomal bifunctional enzymeAdd BLAST723

    Amino acid modifications

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the 'PTM / Processing' section specifies the position and type of each modified residue excluding <a href="http://www.uniprot.org/manual/lipid">lipids</a>, <a href="http://www.uniprot.org/manual/carbohyd">glycans</a> and <a href="http://www.uniprot.org/manual/crosslnk">protein cross-links</a>.<p><a href='/help/mod_res' target='_top'>More...</a></p>Modified residuei38N6-succinyllysineBy similarity1
    Modified residuei165N6-acetyllysine; alternate1 Publication1
    Modified residuei165N6-succinyllysine; alternateBy similarity1
    Modified residuei171N6-acetyllysine1 Publication1
    Modified residuei219N6-acetyllysine; alternateBy similarity1
    Modified residuei219N6-succinyllysine; alternateBy similarity1
    Modified residuei250N6-acetyllysineBy similarity1
    Modified residuei280N6-succinyllysineBy similarity1
    Modified residuei290N6-succinyllysineBy similarity1
    Modified residuei346N6-acetyllysine1 Publication1
    Modified residuei350N6-acetyllysineBy similarity1
    Modified residuei464N6-acetyllysineBy similarity1
    Modified residuei532N6-succinyllysineBy similarity1
    Modified residuei548PhosphothreonineCombined sources1
    Modified residuei577N6-succinyllysineBy similarity1
    Modified residuei584N6-acetyllysine; alternateCombined sources1 Publication1
    Modified residuei584N6-succinyllysine; alternateBy similarity1
    Modified residuei591N6-acetyllysine; alternateBy similarity1
    Modified residuei591N6-succinyllysine; alternateBy similarity1
    Modified residuei710N6-acetyllysine; alternateBy similarity1
    Modified residuei710N6-succinyllysine; alternateBy similarity1
    Modified residuei718PhosphoserineCombined sources1
    Modified residuei722N6-succinyllysineBy similarity1

    <p>This subsection of the <a href="http://www.uniprot.org/help/ptm%5Fprocessing%5Fsection">PTM/processing</a> section describes post-translational modifications (PTMs). This subsection <strong>complements</strong> the information provided at the sequence level or describes modifications for which <strong>position-specific data is not yet available</strong>.<p><a href='/help/post-translational_modification' target='_top'>More...</a></p>Post-translational modificationi

    Acetylated, leading to enhanced enzyme activity. Acetylation is enhanced by up to 80% after treatment either with trichostin A (TSA) or with nicotinamide (NAM) with highest increase on Lys-346. Acetylation and enzyme activity increased by about 1.5% on addition of fatty acids.1 Publication

    Keywords - PTMi

    Acetylation, Phosphoprotein

    Proteomic databases

    Encyclopedia of Proteome Dynamics

    More...
    EPDi
    Q08426

    jPOST - Japan Proteome Standard Repository/Database

    More...
    jPOSTi
    Q08426

    MassIVE - Mass Spectrometry Interactive Virtual Environment

    More...
    MassIVEi
    Q08426

    MaxQB - The MaxQuant DataBase

    More...
    MaxQBi
    Q08426

    PaxDb, a database of protein abundance averages across all three domains of life

    More...
    PaxDbi
    Q08426

    PeptideAtlas

    More...
    PeptideAtlasi
    Q08426

    PRoteomics IDEntifications database

    More...
    PRIDEi
    Q08426

    ProteomicsDB: a multi-organism proteome resource

    More...
    ProteomicsDBi
    58608 [Q08426-1]
    58609 [Q08426-2]

    PTM databases

    iPTMnet integrated resource for PTMs in systems biology context

    More...
    iPTMneti
    Q08426

    Comprehensive resource for the study of protein post-translational modifications (PTMs) in human, mouse and rat.

    More...
    PhosphoSitePlusi
    Q08426

    <p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

    <p>This subsection of the 'Expression' section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms. By default, the information is derived from experiments at the mRNA level, unless specified 'at protein level'.<br></br>Examples: <a href="http://www.uniprot.org/uniprot/P92958#expression">P92958</a>, <a href="http://www.uniprot.org/uniprot/Q8TDN4#expression">Q8TDN4</a>, <a href="http://www.uniprot.org/uniprot/O14734#expression">O14734</a><p><a href='/help/tissue_specificity' target='_top'>More...</a></p>Tissue specificityi

    Liver and kidney. Strongly expressed in the terminal segments of the proximal tubule. Lower amounts seen in the brain.2 Publications

    Gene expression databases

    Bgee dataBase for Gene Expression Evolution

    More...
    Bgeei
    ENSG00000113790, Expressed in liver and 213 other tissues

    ExpressionAtlas, Differential and Baseline Expression

    More...
    ExpressionAtlasi
    Q08426, baseline and differential

    Genevisible search portal to normalized and curated expression data from Genevestigator

    More...
    Genevisiblei
    Q08426, HS

    Organism-specific databases

    Human Protein Atlas

    More...
    HPAi
    ENSG00000113790, Group enriched (kidney, liver)

    <p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

    <p>This subsection of the <a href="http://www.uniprot.org/help/interaction%5Fsection">'Interaction'</a> section provides information about the protein quaternary structure and interaction(s) with other proteins or protein complexes (with the exception of physiological receptor-ligand interactions which are annotated in the <a href="http://www.uniprot.org/help/function%5Fsection">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

    Monomer.

    By similarity

    <p>This subsection of the '<a href="http://www.uniprot.org/help/interaction%5Fsection">Interaction</a>' section provides information about binary protein-protein interactions. The data presented in this section are a quality-filtered subset of binary interactions automatically derived from the <a href="https://www.ebi.ac.uk/intact/">IntAct database</a>. It is updated at every <a href="http://www.uniprot.org/help/synchronization">UniProt release</a>.<p><a href='/help/binary_interactions' target='_top'>More...</a></p>Binary interactionsi

    Q08426
    With#Exp.IntAct
    AARS2 [Q5JTZ9]3EBI-2339219,EBI-308736
    ACTB [P60709]4EBI-2339219,EBI-353944
    ACTG1 [P63261]4EBI-2339219,EBI-351292
    ADARB1 - isoform 4 [P78563-4]3EBI-2339219,EBI-12002366
    ANKRD36B - isoform 4 [Q8N2N9-4]3EBI-2339219,EBI-12170453
    ARMC12 - isoform 2 [Q5T9G4-2]3EBI-2339219,EBI-23667468
    ARMCX3 [Q9UH62]3EBI-2339219,EBI-717832
    BHLHE40 [O14503]3EBI-2339219,EBI-711810
    BOLL - isoform 4 [Q8N9W6-4]3EBI-2339219,EBI-11983447
    C1orf50 [Q9BV19]3EBI-2339219,EBI-2874661
    CAPN3 - isoform IV [P20807-4]3EBI-2339219,EBI-11532021
    CBS [P35520]4EBI-2339219,EBI-740135
    CCDC102B [Q68D86]4EBI-2339219,EBI-10171570
    CCDC13 [Q8IYE1]3EBI-2339219,EBI-10961312
    CDR2L [Q86X02]3EBI-2339219,EBI-11063830
    CEP83 - isoform 2 [Q9Y592-2]3EBI-2339219,EBI-11123098
    CFL2 [Q9Y281]3EBI-2339219,EBI-351218
    CISD2 [Q8N5K1]3EBI-2339219,EBI-1045797
    CLDN18 [P56856]3EBI-2339219,EBI-16354902
    CLDN5 [O00501]3EBI-2339219,EBI-18400628
    CLK2 [P49760]3EBI-2339219,EBI-750020
    CMTM6 [Q9NX76]3EBI-2339219,EBI-1054315
    COQ9 [O75208]3EBI-2339219,EBI-724524
    CYB561 [P49447]3EBI-2339219,EBI-8646596
    CYB561A3 [Q8NBI2]3EBI-2339219,EBI-10269179
    DERL3 [Q96Q80]3EBI-2339219,EBI-12831318
    DES [P17661]6EBI-2339219,EBI-1055572
    DIABLO [Q9NR28]3EBI-2339219,EBI-517508
    EBP [Q15125]3EBI-2339219,EBI-3915253
    EMP1 [P54849]3EBI-2339219,EBI-4319440
    ERGIC1 [Q969X5]3EBI-2339219,EBI-781527
    ERGIC3 [Q9Y282]3EBI-2339219,EBI-781551
    ERVFRD-1 [P60508]3EBI-2339219,EBI-17973325
    FGF14 - isoform 2 [Q92915-2]3EBI-2339219,EBI-12836320
    FUNDC1 [Q8IVP5]4EBI-2339219,EBI-3059266
    GAS8 [O95995]3EBI-2339219,EBI-1052570
    GMCL1 [Q96IK5]3EBI-2339219,EBI-2548508
    GRIPAP1 [Q4V328]3EBI-2339219,EBI-717919
    HID1 [Q8IV36]3EBI-2339219,EBI-743438
    HIP1 [O00291]3EBI-2339219,EBI-473886
    HMG20A [Q9NP66]3EBI-2339219,EBI-740641
    HSD17B11 [Q8NBQ5]3EBI-2339219,EBI-1052304
    HTATIP2 - isoform 3 [Q9BUP3-3]3EBI-2339219,EBI-12937691
    IKZF3 [Q9UKT9]3EBI-2339219,EBI-747204
    JAGN1 [Q8N5M9]3EBI-2339219,EBI-10266796
    KCTD6 [Q8NC69]5EBI-2339219,EBI-2511344
    KCTD9 [Q7L273]4EBI-2339219,EBI-4397613
    KRTAP10-7 [P60409]3EBI-2339219,EBI-10172290
    KRTAP4-11 [Q9BYQ6]3EBI-2339219,EBI-10302392
    LCN2 [P80188]3EBI-2339219,EBI-11911016
    LEPROTL1 [O95214]5EBI-2339219,EBI-750776
    LTBR [P36941]3EBI-2339219,EBI-3509981
    LYPD5 [Q6UWN5]3EBI-2339219,EBI-17200970
    LZTFL1 [Q9NQ48]3EBI-2339219,EBI-2824799
    LZTS2 [Q9BRK4]3EBI-2339219,EBI-741037
    MAB21L3 [Q8N8X9]3EBI-2339219,EBI-10268010
    MID1 [O15344]8EBI-2339219,EBI-2340316
    MTUS2 - isoform 2 [Q5JR59-3]3EBI-2339219,EBI-11522433
    NACC1 [Q96RE7]3EBI-2339219,EBI-7950997
    NECAB2 - isoform 2 [Q7Z6G3-2]3EBI-2339219,EBI-10172876
    OPRM1 - isoform 10 [P35372-10]3EBI-2339219,EBI-12807478
    ORMDL1 [Q9P0S3]3EBI-2339219,EBI-1054848
    PAQR8 [Q8TEZ7]3EBI-2339219,EBI-12847818
    PICK1 [Q9NRD5]3EBI-2339219,EBI-79165
    PNMA1 [Q8ND90]3EBI-2339219,EBI-302345
    PNMA5 [Q96PV4]3EBI-2339219,EBI-10171633
    PPP1R12C [Q9BZL4]3EBI-2339219,EBI-721802
    PRKRA [O75569]3EBI-2339219,EBI-713955
    PRMT5 [O14744]3EBI-2339219,EBI-351098
    PS1TP5BP1 [Q1KLZ0]3EBI-2339219,EBI-9978131
    PSTPIP1 [O43586]3EBI-2339219,EBI-1050964
    PTGES [O14684]3EBI-2339219,EBI-11161398
    REEP6 - isoform 2 [Q96HR9-2]3EBI-2339219,EBI-14065960
    REL - isoform 2 [Q04864-2]3EBI-2339219,EBI-10829018
    RNF4 [P78317]3EBI-2339219,EBI-2340927
    RPRM [Q9NS64]3EBI-2339219,EBI-1052363
    RUSF1 [Q96GQ5]3EBI-2339219,EBI-8636004
    SCARA5 - isoform 2 [Q6ZMJ2-2]3EBI-2339219,EBI-12823227
    SDR16C5 [Q8N3Y7]3EBI-2339219,EBI-3923480
    SFT2D1 [Q8WV19]3EBI-2339219,EBI-2854842
    SGCB [Q16585]3EBI-2339219,EBI-5663627
    SLC14A2 [Q15849]3EBI-2339219,EBI-1573290
    SLC39A2 [Q9NP94]3EBI-2339219,EBI-12898013
    SMIM5 [Q71RC9]3EBI-2339219,EBI-12334905
    SMN2 [Q16637]3EBI-2339219,EBI-395421
    SSMEM1 [Q8WWF3]3EBI-2339219,EBI-17280858
    SSNA1 [O43805]4EBI-2339219,EBI-2515299
    SSX2IP [Q9Y2D8]3EBI-2339219,EBI-2212028
    SYNGR1 - isoform 1B [O43759-2]3EBI-2339219,EBI-12187159
    SYNGR3 [O43761]3EBI-2339219,EBI-11321949
    TMEM121 [Q9BTD3]3EBI-2339219,EBI-12155101
    TMEM17 [Q86X19]3EBI-2339219,EBI-11343485
    TMEM35A [Q53FP2]3EBI-2339219,EBI-11722971
    TNIP1 [Q15025]4EBI-2339219,EBI-357849
    TPP2 [P29144]5EBI-2339219,EBI-1044672
    TRAF1 [Q13077]3EBI-2339219,EBI-359224
    TRAF2 [Q12933]3EBI-2339219,EBI-355744
    TRIM21 [P19474]3EBI-2339219,EBI-81290
    TRIM23 [P36406]3EBI-2339219,EBI-740098
    TRIM27 [P14373]6EBI-2339219,EBI-719493
    TRIM41 [Q8WV44]7EBI-2339219,EBI-725997
    TRIM5 - isoform Gamma [Q9C035-3]3EBI-2339219,EBI-12840050
    TRIM54 [Q9BYV2]3EBI-2339219,EBI-2130429
    TRIM63 [Q969Q1]3EBI-2339219,EBI-5661333
    UBXN1 - isoform 2 [Q04323-2]3EBI-2339219,EBI-11530712
    VAC14 [Q08AM6]3EBI-2339219,EBI-2107455
    ZBTB10 - isoform 3 [Q96DT7-3]3EBI-2339219,EBI-12017160
    ZBTB26 [Q9HCK0]5EBI-2339219,EBI-3918996
    ZBTB8A [Q96BR9]3EBI-2339219,EBI-742740
    ZBTB9 [Q96C00]6EBI-2339219,EBI-395708
    ZCCHC17 [Q9NP64]3EBI-2339219,EBI-746345
    ZRANB1 [Q9UGI0]3EBI-2339219,EBI-527853

    GO - Molecular functioni

    Protein-protein interaction databases

    The Biological General Repository for Interaction Datasets (BioGRID)

    More...
    BioGRIDi
    108282, 114 interactors

    Protein interaction database and analysis system

    More...
    IntActi
    Q08426, 121 interactors

    STRING: functional protein association networks

    More...
    STRINGi
    9606.ENSP00000231887

    Miscellaneous databases

    RNAct, Protein-RNA interaction predictions for model organisms.

    More...
    RNActi
    Q08426, protein

    <p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

    3D structure databases

    SWISS-MODEL Repository - a database of annotated 3D protein structure models

    More...
    SMRi
    Q08426

    Database of comparative protein structure models

    More...
    ModBasei
    Search...

    <p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

    Region

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the 'Family and Domains' section describes a region of interest that cannot be described in other subsections.<p><a href='/help/region' target='_top'>More...</a></p>Regioni1 – 282Enoyl-CoA hydratase / isomeraseAdd BLAST282
    Regioni283 – 5723-hydroxyacyl-CoA dehydrogenaseAdd BLAST290

    Motif

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the 'Family and Domains' section describes a short (usually not more than 20 amino acids) conserved sequence motif of biological significance.<p><a href='/help/motif' target='_top'>More...</a></p>Motifi721 – 723Microbody targeting signal3

    <p>This subsection of the 'Family and domains' section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

    In the N-terminal section; belongs to the enoyl-CoA hydratase/isomerase family.Curated
    In the C-terminal section; belongs to the 3-hydroxyacyl-CoA dehydrogenase family.Curated

    Phylogenomic databases

    evolutionary genealogy of genes: Non-supervised Orthologous Groups

    More...
    eggNOGi
    KOG1683, Eukaryota

    Ensembl GeneTree

    More...
    GeneTreei
    ENSGT00940000157516

    The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

    More...
    HOGENOMi
    CLU_009834_16_3_1

    InParanoid: Eukaryotic Ortholog Groups

    More...
    InParanoidi
    Q08426

    Identification of Orthologs from Complete Genome Data

    More...
    OMAi
    INGYGWP

    Database of Orthologous Groups

    More...
    OrthoDBi
    219667at2759

    Database for complete collections of gene phylogenies

    More...
    PhylomeDBi
    Q08426

    TreeFam database of animal gene trees

    More...
    TreeFami
    TF316708

    Family and domain databases

    Integrated resource of protein families, domains and functional sites

    More...
    InterProi
    View protein in InterPro
    IPR006180, 3-OHacyl-CoA_DH_CS
    IPR006176, 3-OHacyl-CoA_DH_NAD-bd
    IPR006108, 3HC_DH_C
    IPR008927, 6-PGluconate_DH-like_C_sf
    IPR029045, ClpP/crotonase-like_dom_sf
    IPR018376, Enoyl-CoA_hyd/isom_CS
    IPR001753, Enoyl-CoA_hydra/iso
    IPR036291, NAD(P)-bd_dom_sf

    Pfam protein domain database

    More...
    Pfami
    View protein in Pfam
    PF00725, 3HCDH, 2 hits
    PF02737, 3HCDH_N, 1 hit
    PF00378, ECH_1, 1 hit

    Superfamily database of structural and functional annotation

    More...
    SUPFAMi
    SSF48179, SSF48179, 2 hits
    SSF51735, SSF51735, 1 hit
    SSF52096, SSF52096, 1 hit

    PROSITE; a protein domain and family database

    More...
    PROSITEi
    View protein in PROSITE
    PS00067, 3HCDH, 1 hit
    PS00166, ENOYL_COA_HYDRATASE, 1 hit

    <p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence%5Flength">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>. The information is filed in different subsections. The current subsections and their content are listed below:<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequences (2+)i

    <p>This subsection of the <a href="http://www.uniprot.org/help/sequences%5Fsection">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusi: Complete.

    This entry describes 2 <p>This subsection of the 'Sequence' section lists the alternative protein sequences (isoforms) that can be generated from the same gene by a single or by the combination of up to four biological events (alternative promoter usage, alternative splicing, alternative initiation and ribosomal frameshifting). Additionally, this section gives relevant information on each alternative protein isoform. This section is only present in reviewed entries, i.e. in UniProtKB/Swiss-Prot.<p><a href='/help/alternative_products' target='_top'>More...</a></p> isoformsi produced by alternative splicing. AlignAdd to basket

    This entry has 2 described isoforms and 1 potential isoform that is computationally mapped.Show allAlign All

    Isoform 1 (identifier: Q08426-1) [UniParc]FASTAAdd to basket

    This isoform has been chosen as the <div> <p><b>What is the canonical sequence?</b><p><a href='/help/canonical_and_isoforms' target='_top'>More...</a></p>canonicali sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

    « Hide
            10         20         30         40         50
    MAEYTRLHNA LALIRLRNPP VNAISTTLLR DIKEGLQKAV IDHTIKAIVI
    60 70 80 90 100
    CGAEGKFSAG ADIRGFSAPR TFGLTLGHVV DEIQRNEKPV VAAIQGMAFG
    110 120 130 140 150
    GGLELALGCH YRIAHAEAQV GLPEVTLGLL PGARGTQLLP RLTGVPAALD
    160 170 180 190 200
    LITSGRRILA DEALKLGILD KVVNSDPVEE AIRFAQRVSD QPLESRRLCN
    210 220 230 240 250
    KPIQSLPNMD SIFSEALLKM RRQHPGCLAQ EACVRAVQAA VQYPYEVGIK
    260 270 280 290 300
    KEEELFLYLL QSGQARALQY AFFAERKANK WSTPSGASWK TASARPVSSV
    310 320 330 340 350
    GVVGLGTMGR GIVISFARAR IPVIAVDSDK NQLATANKMI TSVLEKEASK
    360 370 380 390 400
    MQQSGHPWSG PKPRLTSSVK ELGGVDLVIE AVFEEMSLKK QVFAELSAVC
    410 420 430 440 450
    KPEAFLCTNT SALDVDEIAS STDRPHLVIG THFFSPAHVM KLLEVIPSQY
    460 470 480 490 500
    SSPTTIATVM NLSKKIKKIG VVVGNCFGFV GNRMLNPYYN QAYFLLEEGS
    510 520 530 540 550
    KPEEVDQVLE EFGFKMGPFR VSDLAGLDVG WKSRKGQGLT GPTLLPGTPA
    560 570 580 590 600
    RKRGNRRYCP IPDVLCELGR FGQKTGKGWY QYDKPLGRIH KPDPWLSKFL
    610 620 630 640 650
    SRYRKTHHIE PRTISQDEIL ERCLYSLINE AFRILGEGIA ASPEHIDVVY
    660 670 680 690 700
    LHGYGWPRHK GGPMFYASTV GLPTVLEKLQ KYYRQNPDIP QLEPSDYLKK
    710 720
    LASQGNPPLK EWQSLAGSPS SKL
    Length:723
    Mass (Da):79,495
    Last modified:February 10, 2009 - v3
    <p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.</p> <p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.</p> <p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).</p> <p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x<sup>64</sup> + x<sup>4</sup> + x<sup>3</sup> + x + 1. The algorithm is described in the ISO 3309 standard. </p> <p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.<br /> <strong>Cyclic redundancy and other checksums</strong><br /> <a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993)</a>)</p> Checksum:iFC3B44B030A7BCBD
    GO
    Isoform 2 (identifier: Q08426-2) [UniParc]FASTAAdd to basket

    The sequence of this isoform differs from the canonical sequence as follows:
         1-96: Missing.

    Show »
    Length:627
    Mass (Da):69,154
    Checksum:iF1F1A30F0E89ED98
    GO

    <p>In eukaryotic reference proteomes, unreviewed entries that are likely to belong to the same gene are computationally mapped, based on gene identifiers from Ensembl, EnsemblGenomes and model organism databases.<p><a href='/help/gene_centric_isoform_mapping' target='_top'>More...</a></p>Computationally mapped potential isoform sequencesi

    There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
    EntryEntry nameProtein names
    Gene namesLengthAnnotation
    C9JJE0C9JJE0_HUMAN
    Peroxisomal bifunctional enzyme
    EHHADH
    76Annotation score:

    Annotation score:1 out of 5

    <p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>

    Experimental Info

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the 'Sequence' section reports difference(s) between the canonical sequence (displayed by default in the entry) and the different sequence submissions merged in the entry. These various submissions may originate from different sequencing projects, different types of experiments, or different biological samples. Sequence conflicts are usually of unknown origin.<p><a href='/help/conflict' target='_top'>More...</a></p>Sequence conflicti117E → D in AAA53289 (PubMed:8188243).Curated1
    Sequence conflicti656 – 657WP → CA in AAA53289 (PubMed:8188243).Curated2
    Sequence conflicti656 – 657WP → CA in AAB19482 (PubMed:15489334).Curated2

    Natural variant

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Natural variantiVAR_0709493E → K in FRTS3; the mutant is mistargeted to mitochondria; results in impaired mitochondrial oxidative phosphorylation and defects in the transport of fluids across the epithelium of renal proximal tubular cells. 1 PublicationCorresponds to variant dbSNP:rs398124646EnsemblClinVar.1
    Natural variantiVAR_05432940V → G1 PublicationCorresponds to variant dbSNP:rs1062551Ensembl.1
    Natural variantiVAR_05433041I → R1 PublicationCorresponds to variant dbSNP:rs1062552Ensembl.1
    Natural variantiVAR_04713275T → I1 PublicationCorresponds to variant dbSNP:rs1062553Ensembl.1
    Natural variantiVAR_047133274A → T1 PublicationCorresponds to variant dbSNP:rs2302819Ensembl.1
    Natural variantiVAR_054331325A → G1 PublicationCorresponds to variant dbSNP:rs1062555Ensembl.1
    Natural variantiVAR_054332598K → T2 PublicationsCorresponds to variant dbSNP:rs1042437Ensembl.1
    Natural variantiVAR_047134606T → P2 PublicationsCorresponds to variant dbSNP:rs1042438Ensembl.1
    Natural variantiVAR_047135685Q → K. Corresponds to variant dbSNP:rs11919970Ensembl.1
    Natural variantiVAR_047136715L → S. Corresponds to variant dbSNP:rs11927618EnsemblClinVar.1

    Alternative sequence

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the 'Sequence' section describes the sequence of naturally occurring alternative protein isoform(s). The changes in the amino acid sequence may be due to alternative splicing, alternative promoter usage, alternative initiation, or ribosomal frameshifting.<p><a href='/help/var_seq' target='_top'>More...</a></p>Alternative sequenceiVSP_0428111 – 96Missing in isoform 2. 1 PublicationAdd BLAST96

    Sequence databases

    Select the link destinations:

    EMBL nucleotide sequence database

    More...
    EMBLi

    GenBank nucleotide sequence database

    More...
    GenBanki

    DNA Data Bank of Japan; a nucleotide sequence database

    More...
    DDBJi
    Links Updated
    L07077 mRNA Translation: AAA53289.1
    AJ427345 AJ427351 Genomic DNA Translation: CAD22483.1
    AK291798 mRNA Translation: BAF84487.1
    AK223460 mRNA Translation: BAD97180.1
    AK301521 mRNA Translation: BAG63025.1
    AC007934 Genomic DNA No translation available.
    AC128680 Genomic DNA No translation available.
    CH471052 Genomic DNA Translation: EAW78229.1
    CH471052 Genomic DNA Translation: EAW78230.1
    BC038948 mRNA Translation: AAH38948.1
    BC110460 mRNA Translation: AAI10461.1
    S50245 mRNA Translation: AAB19482.1

    The Consensus CDS (CCDS) project

    More...
    CCDSi
    CCDS33901.1 [Q08426-1]
    CCDS54694.1 [Q08426-2]

    Protein sequence database of the Protein Information Resource

    More...
    PIRi
    A49613

    NCBI Reference Sequences

    More...
    RefSeqi
    NP_001159887.1, NM_001166415.1 [Q08426-2]
    NP_001957.2, NM_001966.3 [Q08426-1]
    XP_011510819.1, XM_011512517.1

    Genome annotation databases

    Ensembl eukaryotic genome annotation project

    More...
    Ensembli
    ENST00000231887; ENSP00000231887; ENSG00000113790 [Q08426-1]
    ENST00000456310; ENSP00000387746; ENSG00000113790 [Q08426-2]

    Database of genes from NCBI RefSeq genomes

    More...
    GeneIDi
    1962

    KEGG: Kyoto Encyclopedia of Genes and Genomes

    More...
    KEGGi
    hsa:1962

    UCSC genome browser

    More...
    UCSCi
    uc003fpf.3, human [Q08426-1]

    Keywords - Coding sequence diversityi

    Alternative splicing

    <p>This section provides links to proteins that are similar to the protein sequence(s) described in this entry at different levels of sequence identity thresholds (100%, 90% and 50%) based on their membership in UniProt Reference Clusters (<a href="http://www.uniprot.org/help/uniref">UniRef</a>).<p><a href='/help/similar_proteins_section' target='_top'>More...</a></p>Similar proteinsi

    <p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBLi
    GenBanki
    DDBJi
    Links Updated
    L07077 mRNA Translation: AAA53289.1
    AJ427345 AJ427351 Genomic DNA Translation: CAD22483.1
    AK291798 mRNA Translation: BAF84487.1
    AK223460 mRNA Translation: BAD97180.1
    AK301521 mRNA Translation: BAG63025.1
    AC007934 Genomic DNA No translation available.
    AC128680 Genomic DNA No translation available.
    CH471052 Genomic DNA Translation: EAW78229.1
    CH471052 Genomic DNA Translation: EAW78230.1
    BC038948 mRNA Translation: AAH38948.1
    BC110460 mRNA Translation: AAI10461.1
    S50245 mRNA Translation: AAB19482.1
    CCDSiCCDS33901.1 [Q08426-1]
    CCDS54694.1 [Q08426-2]
    PIRiA49613
    RefSeqiNP_001159887.1, NM_001166415.1 [Q08426-2]
    NP_001957.2, NM_001966.3 [Q08426-1]
    XP_011510819.1, XM_011512517.1

    3D structure databases

    SMRiQ08426
    ModBaseiSearch...

    Protein-protein interaction databases

    BioGRIDi108282, 114 interactors
    IntActiQ08426, 121 interactors
    STRINGi9606.ENSP00000231887

    Chemistry databases

    DrugBankiDB00157, NADH
    SwissLipidsiSLP:000000543

    PTM databases

    iPTMnetiQ08426
    PhosphoSitePlusiQ08426

    Genetic variation databases

    BioMutaiEHHADH
    DMDMi223590229

    Proteomic databases

    EPDiQ08426
    jPOSTiQ08426
    MassIVEiQ08426
    MaxQBiQ08426
    PaxDbiQ08426
    PeptideAtlasiQ08426
    PRIDEiQ08426
    ProteomicsDBi58608 [Q08426-1]
    58609 [Q08426-2]

    Protocols and materials databases

    Antibodypedia a portal for validated antibodies

    More...
    Antibodypediai
    33832, 328 antibodies

    The DNASU plasmid repository

    More...
    DNASUi
    1962

    Genome annotation databases

    EnsembliENST00000231887; ENSP00000231887; ENSG00000113790 [Q08426-1]
    ENST00000456310; ENSP00000387746; ENSG00000113790 [Q08426-2]
    GeneIDi1962
    KEGGihsa:1962
    UCSCiuc003fpf.3, human [Q08426-1]

    Organism-specific databases

    Comparative Toxicogenomics Database

    More...
    CTDi
    1962
    DisGeNETi1962

    GeneCards: human genes, protein and diseases

    More...
    GeneCardsi
    EHHADH
    HGNCiHGNC:3247, EHHADH
    HPAiENSG00000113790, Group enriched (kidney, liver)
    MalaCardsiEHHADH
    MIMi607037, gene
    615605, phenotype
    neXtProtiNX_Q08426
    OpenTargetsiENSG00000113790
    Orphaneti300, Bifunctional enzyme deficiency
    3337, Primary Fanconi renotubular syndrome
    PharmGKBiPA27682
    VEuPathDBiHostDB:ENSG00000113790.10

    GenAtlas: human gene database

    More...
    GenAtlasi
    Search...

    Phylogenomic databases

    eggNOGiKOG1683, Eukaryota
    GeneTreeiENSGT00940000157516
    HOGENOMiCLU_009834_16_3_1
    InParanoidiQ08426
    OMAiINGYGWP
    OrthoDBi219667at2759
    PhylomeDBiQ08426
    TreeFamiTF316708

    Enzyme and pathway databases

    UniPathwayiUPA00659
    BioCyciMetaCyc:HS03720-MONOMER
    PathwayCommonsiQ08426
    ReactomeiR-HSA-390247, Beta-oxidation of very long chain fatty acids
    R-HSA-9033241, Peroxisomal protein import
    SABIO-RKiQ08426

    Miscellaneous databases

    BioGRID ORCS database of CRISPR phenotype screens

    More...
    BioGRID-ORCSi
    1962, 7 hits in 999 CRISPR screens

    ChiTaRS: a database of human, mouse and fruit fly chimeric transcripts and RNA-sequencing data

    More...
    ChiTaRSi
    EHHADH, human

    Database of phenotypes from RNA interference screens in Drosophila and Homo sapiens

    More...
    GenomeRNAii
    1962
    PharosiQ08426, Tbio

    Protein Ontology

    More...
    PROi
    PR:Q08426
    RNActiQ08426, protein

    The Stanford Online Universal Resource for Clones and ESTs

    More...
    SOURCEi
    Search...

    Gene expression databases

    BgeeiENSG00000113790, Expressed in liver and 213 other tissues
    ExpressionAtlasiQ08426, baseline and differential
    GenevisibleiQ08426, HS

    Family and domain databases

    InterProiView protein in InterPro
    IPR006180, 3-OHacyl-CoA_DH_CS
    IPR006176, 3-OHacyl-CoA_DH_NAD-bd
    IPR006108, 3HC_DH_C
    IPR008927, 6-PGluconate_DH-like_C_sf
    IPR029045, ClpP/crotonase-like_dom_sf
    IPR018376, Enoyl-CoA_hyd/isom_CS
    IPR001753, Enoyl-CoA_hydra/iso
    IPR036291, NAD(P)-bd_dom_sf
    PfamiView protein in Pfam
    PF00725, 3HCDH, 2 hits
    PF02737, 3HCDH_N, 1 hit
    PF00378, ECH_1, 1 hit
    SUPFAMiSSF48179, SSF48179, 2 hits
    SSF51735, SSF51735, 1 hit
    SSF52096, SSF52096, 1 hit
    PROSITEiView protein in PROSITE
    PS00067, 3HCDH, 1 hit
    PS00166, ENOYL_COA_HYDRATASE, 1 hit

    ProtoNet; Automatic hierarchical classification of proteins

    More...
    ProtoNeti
    Search...

    MobiDB: a database of protein disorder and mobility annotations

    More...
    MobiDBi
    Search...

    <p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

    <p>This subsection of the 'Entry information' section provides a mnemonic identifier for a UniProtKB entry, but it is not a stable identifier. Each reviewed entry is assigned a unique entry name upon integration into UniProtKB/Swiss-Prot.<p><a href='/help/entry_name' target='_top'>More...</a></p>Entry nameiECHP_HUMAN
    <p>This subsection of the 'Entry information' section provides one or more accession number(s). These are stable identifiers and should be used to cite UniProtKB entries. Upon integration into UniProtKB, each entry is assigned a unique accession number, which is called 'Primary (citable) accession number'.<p><a href='/help/accession_numbers' target='_top'>More...</a></p>AccessioniPrimary (citable) accession number: Q08426
    Secondary accession number(s): A8K6Y3
    , B4DWG3, D3DNU0, Q58EZ5
    <p>This subsection of the 'Entry information' section shows the date of integration of the entry into UniProtKB, the date of the last sequence update and the date of the last annotation modification ('Last modified'). The version number for both the entry and the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: October 1, 1994
    Last sequence update: February 10, 2009
    Last modified: April 7, 2021
    This is version 205 of the entry and version 3 of the sequence. See complete history.
    <p>This subsection of the 'Entry information' section indicates whether the entry has been manually annotated and reviewed by UniProtKB curators or not, in other words, if the entry belongs to the Swiss-Prot section of UniProtKB (<strong>reviewed</strong>) or to the computer-annotated TrEMBL section (<strong>unreviewed</strong>).<p><a href='/help/entry_status' target='_top'>More...</a></p>Entry statusiReviewed (UniProtKB/Swiss-Prot)
    Annotation programChordata Protein Annotation Program
    DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

    <p>This section contains any relevant information that doesn't fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

    Keywords - Technical termi

    Reference proteome

    Documents

    1. Human chromosome 3
      Human chromosome 3: entries, gene names and cross-references to MIM
    2. Human entries with genetic variants
      List of human entries with genetic variants
    3. Human variants curated from literature reports
      Index of human variants curated from literature reports
    4. MIM cross-references
      Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
    5. PATHWAY comments
      Index of metabolic and biosynthesis pathways
    6. SIMILARITY comments
      Index of protein domains and families
    UniProt is an ELIXIR core data resource
    Main funding by: National Institutes of Health

    We'd like to inform you that we have updated our Privacy Notice to comply with Europe’s new General Data Protection Regulation (GDPR) that applies since 25 May 2018.

    Do not show this banner again