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Entry version 164 (05 Jun 2019)
Sequence version 1 (01 Mar 2001)
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Protein

Acyl-CoA dehydrogenase family member 9, mitochondrial

Gene

ACAD9

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

Required for mitochondrial complex I assembly (PubMed:20816094, PubMed:24158852). Has a dehydrogenase activity on palmitoyl-CoA (C16:0) and stearoyl-CoA (C18:0). It is three times more active on palmitoyl-CoA than on stearoyl-CoA. However, it does not play a primary role in long-chain fatty acid oxidation in vivo (PubMed:20816094, PubMed:24158852). Has little activity on octanoyl-CoA (C8:0), butyryl-CoA (C4:0) or isovaleryl-CoA (5:0).2 Publications

<p>This subsection of the ‘Function’ section provides information relevant to cofactors. A cofactor is any non-protein substance required for a protein to be catalytically active. Some cofactors are inorganic, such as the metal atoms zinc, iron, and copper in various oxidation states. Others, such as most vitamins, are organic.<p><a href='/help/cofactor' target='_top'>More...</a></p>Cofactori

FADBy similarity

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">Function</a> section is used for enzymes and indicates the residues directly involved in catalysis.<p><a href='/help/act_site' target='_top'>More...</a></p>Active sitei426Proton acceptorBy 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 functionOxidoreductase
LigandFAD, Flavoprotein

Enzyme and pathway databases

Reactome - a knowledgebase of biological pathways and processes

More...
Reactomei
R-HSA-6799198 Complex I biogenesis

SABIO-RK: Biochemical Reaction Kinetics Database

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SABIO-RKi
Q9H845

Chemistry databases

SwissLipids knowledge resource for lipid biology

More...
SwissLipidsi
SLP:000000619

<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_and_taxonomy_section">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:
Acyl-CoA dehydrogenase family member 9, mitochondrial (EC:1.3.99.-)
Short name:
ACAD-9
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">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:ACAD9
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">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_and_taxonomy_section">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_and_taxonomy_section">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_and_taxonomy_section">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_manual">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:21497 ACAD9

Online Mendelian Inheritance in Man (OMIM)

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MIMi
611103 gene

neXtProt; the human protein knowledge platform

More...
neXtProti
NX_Q9H845

<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

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte & Seán O’Donoghue; Source: COMPARTMENTS

Keywords - Cellular componenti

Mitochondrion

<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

Acyl-CoA dehydrogenase family, member 9, deficiency (ACAD9 deficiency)8 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionAn autosomal recessive metabolic disorder associated with mitochondrial complex I deficiency, resulting in multisystemic and variable manifestations. Clinical features include infantile onset of acute metabolic acidosis, Reye-like episodes (brain edema and vomiting that may rapidly progress to seizures, coma and death), exercise intolerance, hypertrophic cardiomyopathy, liver failure, muscle weakness, and neurologic dysfunction.
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_07189244F → I in ACAD9 deficiency. 1 PublicationCorresponds to variant dbSNP:rs387907041EnsemblClinVar.1
Natural variantiVAR_071893127R → K in ACAD9 deficiency. 1 Publication1
Natural variantiVAR_071894193R → W in ACAD9 deficiency; unknown pathological significance. 1 PublicationCorresponds to variant dbSNP:rs377547811Ensembl.1
Natural variantiVAR_071895220A → V in ACAD9 deficiency. 1 Publication1
Natural variantiVAR_071896234S → F in ACAD9 deficiency; unknown pathological significance. 1 Publication1
Natural variantiVAR_071897266R → Q in ACAD9 deficiency. 1 PublicationCorresponds to variant dbSNP:rs387907042EnsemblClinVar.1
Natural variantiVAR_076177271C → G in ACAD9 deficiency. 1 Publication1
Natural variantiVAR_071898303G → S in ACAD9 deficiency; unknown pathological significance. 1 PublicationCorresponds to variant dbSNP:rs143383023Ensembl.1
Natural variantiVAR_071899326A → T in ACAD9 deficiency; unknown pathological significance. 1 PublicationCorresponds to variant dbSNP:rs115532916EnsemblClinVar.1
Natural variantiVAR_076178384V → M in ACAD9 deficiency. 1 PublicationCorresponds to variant dbSNP:rs1447947184Ensembl.1
Natural variantiVAR_071900413E → K in ACAD9 deficiency; unknown pathological significance. 1 PublicationCorresponds to variant dbSNP:rs149753643EnsemblClinVar.1
Natural variantiVAR_071901414R → C in ACAD9 deficiency. 1 PublicationCorresponds to variant dbSNP:rs777282696EnsemblClinVar.1
Natural variantiVAR_071902417R → C in ACAD9 deficiency. 1 PublicationCorresponds to variant dbSNP:rs368949613EnsemblClinVar.1
Natural variantiVAR_071903469R → W in ACAD9 deficiency. 1 PublicationCorresponds to variant dbSNP:rs139145143EnsemblClinVar.1
Natural variantiVAR_071904518R → H in ACAD9 deficiency. 1 PublicationCorresponds to variant dbSNP:rs781149699Ensembl.1
Natural variantiVAR_071905532R → W in ACAD9 deficiency. 3 PublicationsCorresponds to variant dbSNP:rs377022708EnsemblClinVar.1
Natural variantiVAR_076179606L → H in ACAD9 deficiency. 1 Publication1

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/manual/pathology_and_biotech_section">'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>Mutagenesisi426E → Q: Catalytically inactive. Does not affect mitochondrial complex I assembly. 1 Publication1

Keywords - Diseasei

Disease mutation, Primary mitochondrial disease

Organism-specific databases

DisGeNET

More...
DisGeNETi
28976

MalaCards human disease database

More...
MalaCardsi
ACAD9
MIMi611126 phenotype

Open Targets

More...
OpenTargetsi
ENSG00000177646

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

More...
Orphaneti
99901 Acyl-CoA dehydrogenase 9 deficiency

The Pharmacogenetics and Pharmacogenomics Knowledge Base

More...
PharmGKBi
PA134900655

Polymorphism and mutation databases

BioMuta curated single-nucleotide variation and disease association database

More...
BioMutai
ACAD9

Domain mapping of disease mutations (DMDM)

More...
DMDMi
32469596

<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 transit peptide.<p><a href='/help/transit' target='_top'>More...</a></p>Transit peptidei1 – ?MitochondrionSequence analysis
<p>This subsection of the ‘PTM / Processing’ section describes the extent of a polypeptide chain in the mature protein following processing.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_0000000524? – 621Acyl-CoA dehydrogenase family member 9, mitochondrial

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 residuei41N6-acetyllysineCombined sources1
Modified residuei92N6-succinyllysineBy similarity1
Modified residuei478PhosphothreonineBy similarity1
Modified residuei521N6-acetyllysine; alternateBy similarity1
Modified residuei521N6-succinyllysine; alternateBy similarity1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

Encyclopedia of Proteome Dynamics

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EPDi
Q9H845

jPOST - Japan Proteome Standard Repository/Database

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jPOSTi
Q9H845

MaxQB - The MaxQuant DataBase

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MaxQBi
Q9H845

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

More...
PaxDbi
Q9H845

PeptideAtlas

More...
PeptideAtlasi
Q9H845

PRoteomics IDEntifications database

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PRIDEi
Q9H845

ProteomicsDB human proteome resource

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ProteomicsDBi
81177

PTM databases

iPTMnet integrated resource for PTMs in systems biology context

More...
iPTMneti
Q9H845

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

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PhosphoSitePlusi
Q9H845

SwissPalm database of S-palmitoylation events

More...
SwissPalmi
Q9H845

<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

Ubiquitously expressed in most normal human tissues and cancer cell lines with high level of expression in heart, skeletal muscles, brain, kidney and liver.1 Publication

Gene expression databases

Bgee dataBase for Gene Expression Evolution

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Bgeei
ENSG00000177646 Expressed in 197 organ(s), highest expression level in upper arm skin

ExpressionAtlas, Differential and Baseline Expression

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ExpressionAtlasi
Q9H845 baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

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Genevisiblei
Q9H845 HS

Organism-specific databases

Human Protein Atlas

More...
HPAi
HPA037716
HPA046720

<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_section">'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_section">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

Part of the mitochondrial complex I assembly (MCIA) complex. The complex comprises at least TMEM126B, NDUFAF1, ECSIT, and ACAD9 (By similarity). Interacts with NDUFAF1 and ECSIT (PubMed:20816094).By similarity1 Publication

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGrid)

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BioGridi
118799, 83 interactors

Database of interacting proteins

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DIPi
DIP-53699N

Protein interaction database and analysis system

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IntActi
Q9H845, 51 interactors

Molecular INTeraction database

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MINTi
Q9H845

STRING: functional protein association networks

More...
STRINGi
9606.ENSP00000312618

<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

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SMRi
Q9H845

Database of comparative protein structure models

More...
ModBasei
Search...

MobiDB: a database of protein disorder and mobility annotations

More...
MobiDBi
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

<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

Belongs to the acyl-CoA dehydrogenase family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

More...
eggNOGi
KOG0137 Eukaryota
COG1960 LUCA

Ensembl GeneTree

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GeneTreei
ENSGT00940000157312

The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

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HOGENOMi
HOG000131665

InParanoid: Eukaryotic Ortholog Groups

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InParanoidi
Q9H845

KEGG Orthology (KO)

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KOi
K15980

Identification of Orthologs from Complete Genome Data

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OMAi
GMMDRPG

Database of Orthologous Groups

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OrthoDBi
819314at2759

Database for complete collections of gene phylogenies

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PhylomeDBi
Q9H845

TreeFam database of animal gene trees

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TreeFami
TF105053

Family and domain databases

Gene3D Structural and Functional Annotation of Protein Families

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Gene3Di
1.10.540.10, 1 hit

Integrated resource of protein families, domains and functional sites

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InterProi
View protein in InterPro
IPR006089 Acyl-CoA_DH_CS
IPR006091 Acyl-CoA_Oxase/DH_cen-dom
IPR036250 AcylCo_DH-like_C
IPR009075 AcylCo_DH/oxidase_C
IPR013786 AcylCoA_DH/ox_N
IPR037069 AcylCoA_DH/ox_N_sf
IPR009100 AcylCoA_DH/oxidase_NM_dom

Pfam protein domain database

More...
Pfami
View protein in Pfam
PF00441 Acyl-CoA_dh_1, 1 hit
PF02770 Acyl-CoA_dh_M, 1 hit
PF02771 Acyl-CoA_dh_N, 1 hit

Superfamily database of structural and functional annotation

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SUPFAMi
SSF47203 SSF47203, 2 hits
SSF56645 SSF56645, 1 hit

PROSITE; a protein domain and family database

More...
PROSITEi
View protein in PROSITE
PS00072 ACYL_COA_DH_1, 1 hit
PS00073 ACYL_COA_DH_2, 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_length">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>Sequence (1+)i

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">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.

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is in its mature form or if it represents the precursor.<p><a href='/help/sequence_processing' target='_top'>More...</a></p>Sequence processingi: The displayed sequence is further processed into a mature form.

This entry has 1 described isoform and 6 potential isoforms that are computationally mapped.Show allAlign All

Q9H845-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MSGCGLFLRT TAAARACRGL VVSTANRRLL RTSPPVRAFA KELFLGKIKK
60 70 80 90 100
KEVFPFPEVS QDELNEINQF LGPVEKFFTE EVDSRKIDQE GKIPDETLEK
110 120 130 140 150
LKSLGLFGLQ VPEEYGGLGF SNTMYSRLGE IISMDGSITV TLAAHQAIGL
160 170 180 190 200
KGIILAGTEE QKAKYLPKLA SGEHIAAFCL TEPASGSDAA SIRSRATLSE
210 220 230 240 250
DKKHYILNGS KVWITNGGLA NIFTVFAKTE VVDSDGSVKD KITAFIVERD
260 270 280 290 300
FGGVTNGKPE DKLGIRGSNT CEVHFENTKI PVENILGEVG DGFKVAMNIL
310 320 330 340 350
NSGRFSMGSV VAGLLKRLIE MTAEYACTRK QFNKRLSEFG LIQEKFALMA
360 370 380 390 400
QKAYVMESMT YLTAGMLDQP GFPDCSIEAA MVKVFSSEAA WQCVSEALQI
410 420 430 440 450
LGGLGYTRDY PYERILRDTR ILLIFEGTNE ILRMYIALTG LQHAGRILTT
460 470 480 490 500
RIHELKQAKV STVMDTVGRR LRDSLGRTVD LGLTGNHGVV HPSLADSANK
510 520 530 540 550
FEENTYCFGR TVETLLLRFG KTIMEEQLVL KRVANILINL YGMTAVLSRA
560 570 580 590 600
SRSIRIGLRN HDHEVLLANT FCVEAYLQNL FSLSQLDKYA PENLDEQIKK
610 620
VSQQILEKRA YICAHPLDRT C
Length:621
Mass (Da):68,760
Last modified:March 1, 2001 - v1
<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:i064BCE0378877F54
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 are 6 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
D6RCD8D6RCD8_HUMAN
Acyl-CoA dehydrogenase family membe...
ACAD9
173Annotation 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>
H0Y8Z9H0Y8Z9_HUMAN
Acyl-CoA dehydrogenase family membe...
ACAD9
384Annotation 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>
D6R9Z3D6R9Z3_HUMAN
Acyl-CoA dehydrogenase family membe...
ACAD9
99Annotation 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>
D6RGK6D6RGK6_HUMAN
Acyl-CoA dehydrogenase family membe...
ACAD9
69Annotation 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>
D6RJA8D6RJA8_HUMAN
Acyl-CoA dehydrogenase family membe...
ACAD9
93Annotation 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>
D6RDK9D6RDK9_HUMAN
Acyl-CoA dehydrogenase family membe...
ACAD9
93Annotation 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 conflicti397A → V in AAL56011 (PubMed:12359260).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_07189244F → I in ACAD9 deficiency. 1 PublicationCorresponds to variant dbSNP:rs387907041EnsemblClinVar.1
Natural variantiVAR_071893127R → K in ACAD9 deficiency. 1 Publication1
Natural variantiVAR_071894193R → W in ACAD9 deficiency; unknown pathological significance. 1 PublicationCorresponds to variant dbSNP:rs377547811Ensembl.1
Natural variantiVAR_071895220A → V in ACAD9 deficiency. 1 Publication1
Natural variantiVAR_071896234S → F in ACAD9 deficiency; unknown pathological significance. 1 Publication1
Natural variantiVAR_071897266R → Q in ACAD9 deficiency. 1 PublicationCorresponds to variant dbSNP:rs387907042EnsemblClinVar.1
Natural variantiVAR_076177271C → G in ACAD9 deficiency. 1 Publication1
Natural variantiVAR_071898303G → S in ACAD9 deficiency; unknown pathological significance. 1 PublicationCorresponds to variant dbSNP:rs143383023Ensembl.1
Natural variantiVAR_071899326A → T in ACAD9 deficiency; unknown pathological significance. 1 PublicationCorresponds to variant dbSNP:rs115532916EnsemblClinVar.1
Natural variantiVAR_076178384V → M in ACAD9 deficiency. 1 PublicationCorresponds to variant dbSNP:rs1447947184Ensembl.1
Natural variantiVAR_071900413E → K in ACAD9 deficiency; unknown pathological significance. 1 PublicationCorresponds to variant dbSNP:rs149753643EnsemblClinVar.1
Natural variantiVAR_071901414R → C in ACAD9 deficiency. 1 PublicationCorresponds to variant dbSNP:rs777282696EnsemblClinVar.1
Natural variantiVAR_071902417R → C in ACAD9 deficiency. 1 PublicationCorresponds to variant dbSNP:rs368949613EnsemblClinVar.1
Natural variantiVAR_071903469R → W in ACAD9 deficiency. 1 PublicationCorresponds to variant dbSNP:rs139145143EnsemblClinVar.1
Natural variantiVAR_033459477R → Q. Corresponds to variant dbSNP:rs4494951EnsemblClinVar.1
Natural variantiVAR_071904518R → H in ACAD9 deficiency. 1 PublicationCorresponds to variant dbSNP:rs781149699Ensembl.1
Natural variantiVAR_071905532R → W in ACAD9 deficiency. 3 PublicationsCorresponds to variant dbSNP:rs377022708EnsemblClinVar.1
Natural variantiVAR_076179606L → H in ACAD9 deficiency. 1 Publication1

Sequence databases

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EMBL nucleotide sequence database

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EMBLi

GenBank nucleotide sequence database

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GenBanki

DNA Data Bank of Japan; a nucleotide sequence database

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DDBJi
Links Updated
AF327351 mRNA Translation: AAL56011.1
AK024012 mRNA Translation: BAB14775.1
CH471052 Genomic DNA Translation: EAW79295.1
CH471052 Genomic DNA Translation: EAW79296.1
BC013354 mRNA Translation: AAH13354.1
BC007970 mRNA Translation: AAH07970.1

The Consensus CDS (CCDS) project

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CCDSi
CCDS3053.1

Protein sequence database of the Protein Information Resource

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PIRi
JC7892

NCBI Reference Sequences

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RefSeqi
NP_054768.2, NM_014049.4

Genome annotation databases

Ensembl eukaryotic genome annotation project

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Ensembli
ENST00000308982; ENSP00000312618; ENSG00000177646

Database of genes from NCBI RefSeq genomes

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GeneIDi
28976

KEGG: Kyoto Encyclopedia of Genes and Genomes

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KEGGi
hsa:28976

UCSC genome browser

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UCSCi
uc003ela.5 human

Keywords - Coding sequence diversityi

Polymorphism

<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
AF327351 mRNA Translation: AAL56011.1
AK024012 mRNA Translation: BAB14775.1
CH471052 Genomic DNA Translation: EAW79295.1
CH471052 Genomic DNA Translation: EAW79296.1
BC013354 mRNA Translation: AAH13354.1
BC007970 mRNA Translation: AAH07970.1
CCDSiCCDS3053.1
PIRiJC7892
RefSeqiNP_054768.2, NM_014049.4

3D structure databases

SMRiQ9H845
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi118799, 83 interactors
DIPiDIP-53699N
IntActiQ9H845, 51 interactors
MINTiQ9H845
STRINGi9606.ENSP00000312618

Chemistry databases

SwissLipidsiSLP:000000619

PTM databases

iPTMnetiQ9H845
PhosphoSitePlusiQ9H845
SwissPalmiQ9H845

Polymorphism and mutation databases

BioMutaiACAD9
DMDMi32469596

Proteomic databases

EPDiQ9H845
jPOSTiQ9H845
MaxQBiQ9H845
PaxDbiQ9H845
PeptideAtlasiQ9H845
PRIDEiQ9H845
ProteomicsDBi81177

Protocols and materials databases

The DNASU plasmid repository

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DNASUi
28976
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000308982; ENSP00000312618; ENSG00000177646
GeneIDi28976
KEGGihsa:28976
UCSCiuc003ela.5 human

Organism-specific databases

Comparative Toxicogenomics Database

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CTDi
28976
DisGeNETi28976

GeneCards: human genes, protein and diseases

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GeneCardsi
ACAD9

H-Invitational Database, human transcriptome db

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H-InvDBi
HIX0003659
HGNCiHGNC:21497 ACAD9
HPAiHPA037716
HPA046720
MalaCardsiACAD9
MIMi611103 gene
611126 phenotype
neXtProtiNX_Q9H845
OpenTargetsiENSG00000177646
Orphaneti99901 Acyl-CoA dehydrogenase 9 deficiency
PharmGKBiPA134900655

GenAtlas: human gene database

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GenAtlasi
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Phylogenomic databases

eggNOGiKOG0137 Eukaryota
COG1960 LUCA
GeneTreeiENSGT00940000157312
HOGENOMiHOG000131665
InParanoidiQ9H845
KOiK15980
OMAiGMMDRPG
OrthoDBi819314at2759
PhylomeDBiQ9H845
TreeFamiTF105053

Enzyme and pathway databases

ReactomeiR-HSA-6799198 Complex I biogenesis
SABIO-RKiQ9H845

Miscellaneous databases

The Gene Wiki collection of pages on human genes and proteins

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GeneWikii
ACAD9

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

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GenomeRNAii
28976

Protein Ontology

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PROi
PR:Q9H845

The Stanford Online Universal Resource for Clones and ESTs

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SOURCEi
Search...

Gene expression databases

BgeeiENSG00000177646 Expressed in 197 organ(s), highest expression level in upper arm skin
ExpressionAtlasiQ9H845 baseline and differential
GenevisibleiQ9H845 HS

Family and domain databases

Gene3Di1.10.540.10, 1 hit
InterProiView protein in InterPro
IPR006089 Acyl-CoA_DH_CS
IPR006091 Acyl-CoA_Oxase/DH_cen-dom
IPR036250 AcylCo_DH-like_C
IPR009075 AcylCo_DH/oxidase_C
IPR013786 AcylCoA_DH/ox_N
IPR037069 AcylCoA_DH/ox_N_sf
IPR009100 AcylCoA_DH/oxidase_NM_dom
PfamiView protein in Pfam
PF00441 Acyl-CoA_dh_1, 1 hit
PF02770 Acyl-CoA_dh_M, 1 hit
PF02771 Acyl-CoA_dh_N, 1 hit
SUPFAMiSSF47203 SSF47203, 2 hits
SSF56645 SSF56645, 1 hit
PROSITEiView protein in PROSITE
PS00072 ACYL_COA_DH_1, 1 hit
PS00073 ACYL_COA_DH_2, 1 hit

ProtoNet; Automatic hierarchical classification of proteins

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ProtoNeti
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<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 nameiACAD9_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: Q9H845
Secondary accession number(s): D3DNB8, Q8WXX3
<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_and_isoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 3, 2003
Last sequence update: March 1, 2001
Last modified: June 5, 2019
This is version 164 of the entry and version 1 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

Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. Human chromosome 3
    Human chromosome 3: entries, gene names and cross-references to MIM
  3. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  4. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  5. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
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