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UniProtKB - Q13057 (COASY_HUMAN)

Entry version 200 (02 Dec 2020)
Sequence version 4 (27 Jun 2003)
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Protein

Bifunctional coenzyme A synthase

Gene

COASY

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

Bifunctional enzyme that catalyzes the fourth and fifth sequential steps of CoA biosynthetic pathway. The fourth reaction is catalyzed by the phosphopantetheine adenylyltransferase, coded by the coaD domain; the fifth reaction is catalyzed by the dephospho-CoA kinase, coded by the coaE domain. May act as a point of CoA biosynthesis regulation.2 Publications

<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 '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=7.6 mM for 4'-phosphopantetheine
  2. KM=145 mM for ATP (in the PPAT reaction)
  3. KM=16.7 mM for dephospho-CoA
  4. KM=34.4 mM for ATP (in the DPCK reaction)

    <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: coenzyme A biosynthesis

    This protein is involved in step 4 and 5 of the subpathway that synthesizes CoA from (R)-pantothenate.
    Proteins known to be involved in the 5 steps of the subpathway in this organism are:
    1. Pantothenate kinase 1 (PANK1), Pantothenate kinase 3 (PANK3), Pantothenate kinase 2, mitochondrial (PANK2)
    2. Phosphopantothenate--cysteine ligase (PPCS)
    3. Phosphopantothenoylcysteine decarboxylase (PPCDC)
    4. Bifunctional coenzyme A synthase (COASY)
    5. Bifunctional coenzyme A synthase (COASY)
    This subpathway is part of the pathway coenzyme A biosynthesis, which is itself part of Cofactor biosynthesis.
    View all proteins of this organism that are known to be involved in the subpathway that synthesizes CoA from (R)-pantothenate, the pathway coenzyme A biosynthesis and in Cofactor biosynthesis.

    Regions

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the <a href="http://www.uniprot.org/help/function%5Fsection">Function</a> section describes a region in the protein which binds nucleotide phosphates. It always involves more than one amino acid and includes all residues involved in nucleotide-binding.<p><a href='/help/np_bind' target='_top'>More...</a></p>Nucleotide bindingi365 – 372ATPSequence analysis8

    <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

    Complete GO annotation on QuickGO ...

    GO - Biological processi

    Complete GO annotation on QuickGO ...

    <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 functionKinase, Multifunctional enzyme, Nucleotidyltransferase, Transferase
    Biological processCoenzyme A biosynthesis
    LigandATP-binding, Nucleotide-binding

    Enzyme and pathway databases

    BioCyc Collection of Pathway/Genome Databases

    More...    BioCyci    		MetaCyc:HS00931-MONOMER

    BRENDA Comprehensive Enzyme Information System

    More...    BRENDAi    		2.7.1.24, 2681
    2.7.7.3, 2681

    Pathway Commons web resource for biological pathway data

    More...    PathwayCommonsi    		Q13057

    Reactome - a knowledgebase of biological pathways and processes

    More...    Reactomei    		R-HSA-196783, Coenzyme A biosynthesis

    SABIO-RK: Biochemical Reaction Kinetics Database

    More...    SABIO-RKi    		Q13057

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

    More...    UniPathwayi    		UPA00241;UER00355
    UPA00241;UER00356

    <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:
    Bifunctional coenzyme A synthase
    Short name:
    CoA synthase
    Alternative name(s):
    NBP
    POV-2
    Including the following 2 domains:
    Phosphopantetheine adenylyltransferase (EC:2.7.7.3)
    Alternative name(s):
    Dephospho-CoA pyrophosphorylase
    Pantetheine-phosphate adenylyltransferase
    Short name:
    PPAT
    Dephospho-CoA kinase (EC:2.7.1.241 Publication)
    Short name:
    DPCK
    Alternative name(s):
    Dephosphocoenzyme A kinase
    Short name:
    DPCOAK
    <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:COASY
    ORF Names:PSEC0106
    <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 lineageiEukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo
    <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 17

    Organism-specific databases

    Eukaryotic Pathogen and Host Database Resources

    More...    EuPathDBi    		HostDB:ENSG00000068120.14

    Human Gene Nomenclature Database

    More...    HGNCi    		HGNC:29932, COASY

    Online Mendelian Inheritance in Man (OMIM)

    More...    MIMi    		609855, gene

    neXtProt; the human protein knowledge platform

    More...    neXtProti    		NX_Q13057

    <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

    Cytoplasm, 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

    Neurodegeneration with brain iron accumulation 6 (NBIA6)2 Publications
    The disease is caused by mutations affecting the gene represented in this entry.
    Disease descriptionA neurodegenerative disorder associated with iron accumulation in the brain, primarily in the basal ganglia. It is characterized by progressive motor and cognitive dysfunction beginning in childhood or young adulthood. Patients show extrapyramidal motor signs, such as spasticity, dystonia, and parkinsonism.
    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_08222259 – 564Missing in NBIA6; reduced protein abundance. 1 PublicationAdd BLAST506
    Natural variantiVAR_082223214A → V in NBIA6; unknown pathological significance. 1 Publication1
    Natural variantiVAR_070975499R → C in NBIA6; reduced protein abundance; loss of dephospho-CoA kinase activity. 2 PublicationsCorresponds to variant dbSNP:rs140709867EnsemblClinVar.1
    Pontocerebellar hypoplasia 12 (PCH12)1 Publication
    The disease is caused by mutations affecting the gene represented in this entry.
    Disease descriptionA form of pontocerebellar hypoplasia, a disorder characterized by structural defects of the pons and cerebellum, evident upon brain imaging. PCH12 is an autosomal recessive form characterized by onset in utero and death in infancy. Brain imaging shows microcephaly, cerebellar hypoplasia, micrognathia, and multiple contractures.
    Related information in OMIM

    Keywords - Diseasei

    Disease mutation, Neurodegeneration

    Organism-specific databases

    DisGeNET

    More...    DisGeNETi    		80347

    MalaCards human disease database

    More...    MalaCardsi    		COASY
    MIMi615643, phenotype
    618266, phenotype

    Open Targets

    More...    OpenTargetsi    		ENSG00000068120

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

    More...    Orphaneti    		397725, COASY protein-associated neurodegeneration

    The Pharmacogenetics and Pharmacogenomics Knowledge Base

    More...    PharmGKBi    		PA134867942

    Miscellaneous databases

    Pharos NIH Druggable Genome Knowledgebase

    More...    Pharosi    		Q13057, Tbio

    Chemistry databases

    ChEMBL database of bioactive drug-like small molecules

    More...    ChEMBLi    		CHEMBL4105867

    Polymorphism and mutation databases

    BioMuta curated single-nucleotide variation and disease association database

    More...    BioMutai    		COASY

    Domain mapping of disease mutations (DMDM)

    More...    DMDMi    		32363505

    <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_00001730391 – 564Bifunctional coenzyme A synthaseAdd BLAST564

    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 residuei178PhosphoserineCombined sources1
    Modified residuei183PhosphoserineCombined sources1

    Keywords - PTMi

    Phosphoprotein

    Proteomic databases

    Encyclopedia of Proteome Dynamics

    More...    EPDi    		Q13057

    jPOST - Japan Proteome Standard Repository/Database

    More...    jPOSTi    		Q13057

    MassIVE - Mass Spectrometry Interactive Virtual Environment

    More...    MassIVEi    		Q13057

    MaxQB - The MaxQuant DataBase

    More...    MaxQBi    		Q13057

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

    More...    PaxDbi    		Q13057

    PeptideAtlas

    More...    PeptideAtlasi    		Q13057

    PRoteomics IDEntifications database

    More...    PRIDEi    		Q13057

    ProteomicsDB: a multi-organism proteome resource

    More...    ProteomicsDBi    		59124 [Q13057-1]
    59125 [Q13057-2]

    PTM databases

    iPTMnet integrated resource for PTMs in systems biology context

    More...    iPTMneti    		Q13057

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

    More...    PhosphoSitePlusi    		Q13057

    <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

    Expressed in all tissues examined including brain, heart, skeletal muscle, colon, thymus, spleen, kidney, liver, small intestine, placenta, lung and peripheral blood leukocyte. Lowest expression in peripheral blood leukocytes and highest in kidney and liver. Isoform 2 is expressed mainly in the brain.1 Publication

    Gene expression databases

    Bgee dataBase for Gene Expression Evolution

    More...    Bgeei    		ENSG00000068120, Expressed in body of stomach and 229 other tissues

    ExpressionAtlas, Differential and Baseline Expression

    More...    ExpressionAtlasi    		Q13057, baseline and differential

    Genevisible search portal to normalized and curated expression data from Genevestigator

    More...    Genevisiblei    		Q13057, HS

    Organism-specific databases

    Human Protein Atlas

    More...    HPAi    		ENSG00000068120, Low tissue specificity

    <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.

    1 Publication

    <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

    Hide details

    Protein-protein interaction databases

    The Biological General Repository for Interaction Datasets (BioGRID)

    More...    BioGRIDi    		123254, 46 interactors

    Protein interaction database and analysis system

    More...    IntActi    		Q13057, 19 interactors

    Molecular INTeraction database

    More...    MINTi    		Q13057

    STRING: functional protein association networks

    More...    STRINGi    		9606.ENSP00000464814

    Chemistry databases

    BindingDB database of measured binding affinities

    More...    BindingDBi    		Q13057

    Miscellaneous databases

    RNAct, Protein-RNA interaction predictions for model organisms.

    More...    RNActi    		Q13057, protein

    <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

    Domains and Repeats

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the <a href="http://www.uniprot.org/help/family%5Fand%5Fdomains%5Fsection">Family and Domains</a> section describes the position and type of a domain, which is defined as a specific combination of secondary structures organized into a characteristic three-dimensional structure or fold.<p><a href='/help/domain' target='_top'>More...</a></p>Domaini360 – 563DPCKAdd BLAST204

    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>Regioni180 – 358Phosphopantetheine adenylyltransferaseAdd BLAST179

    <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 central section; belongs to the eukaryotic CoaD family.Curated

    Phylogenomic databases

    evolutionary genealogy of genes: Non-supervised Orthologous Groups

    More...    eggNOGi    		KOG3220, Eukaryota
    KOG3351, Eukaryota

    Ensembl GeneTree

    More...    GeneTreei    		ENSGT00550000075078

    The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

    More...    HOGENOMi    		CLU_027827_1_0_1

    InParanoid: Eukaryotic Ortholog Groups

    More...    InParanoidi    		Q13057

    Identification of Orthologs from Complete Genome Data

    More...    OMAi    		VIDCDKV

    Database of Orthologous Groups

    More...    OrthoDBi    		1543581at2759

    Database for complete collections of gene phylogenies

    More...    PhylomeDBi    		Q13057

    Family and domain databases

    Gene3D Structural and Functional Annotation of Protein Families

    More...    Gene3Di    		3.40.50.620, 1 hit

    HAMAP database of protein families

    More...    HAMAPi    		MF_00376, Dephospho_CoA_kinase, 1 hit

    Integrated resource of protein families, domains and functional sites

    More...    InterProi    		View protein in InterPro
    IPR004821, Cyt_trans-like
    IPR001977, Depp_CoAkinase
    IPR027417, P-loop_NTPase
    IPR014729, Rossmann-like_a/b/a_fold

    Pfam protein domain database

    More...    Pfami    		View protein in Pfam
    PF01121, CoaE, 1 hit
    PF01467, CTP_transf_like, 1 hit

    Superfamily database of structural and functional annotation

    More...    SUPFAMi    		SSF52540, SSF52540, 1 hit

    TIGRFAMs; a protein family database

    More...    TIGRFAMsi    		TIGR00152, TIGR00152, 1 hit

    PROSITE; a protein domain and family database

    More...    PROSITEi    		View protein in PROSITE
    PS51219, DPCK, 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 7 potential isoforms that are computationally mapped.Show allAlign All

    Isoform 1 (identifier: Q13057-1) [UniParc]FASTAAdd to basket
    Also known as: CoASy alpha

    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
    MAVFRSGLLV LTTPLASLAP RLASILTSAA RLVNHTLYVH LQPGMSLEGP 
            60         70         80         90        100
    AQPQSSPVQA TFEVLDFITH LYAGADVHRH LDVRILLTNI RTKSTFLPPL 
           110        120        130        140        150
    PTSVQNLAHP PEVVLTDFQT LDGSQYNPVK QQLVRYATSC YSCCPRLASV 
           160        170        180        190        200
    LLYSDYGIGE VPVEPLDVPL PSTIRPASPV AGSPKQPVRG YYRGAVGGTF 
           210        220        230        240        250
    DRLHNAHKVL LSVACILAQE QLVVGVADKD LLKSKLLPEL LQPYTERVEH 
           260        270        280        290        300
    LSEFLVDIKP SLTFDVIPLL DPYGPAGSDP SLEFLVVSEE TYRGGMAINR 
           310        320        330        340        350
    FRLENDLEEL ALYQIQLLKD LRHTENEEDK VSSSSFRQRM LGNLLRPPYE 
           360        370        380        390        400
    RPELPTCLYV IGLTGISGSG KSSIAQRLKG LGAFVIDSDH LGHRAYAPGG 
           410        420        430        440        450
    PAYQPVVEAF GTDILHKDGI INRKVLGSRV FGNKKQLKIL TDIMWPIIAK 
           460        470        480        490        500
    LAREEMDRAV AEGKRVCVID AAVLLEAGWQ NLVHEVWTAV IPETEAVRRI 
           510        520        530        540        550
    VERDGLSEAA AQSRLQSQMS GQQLVEQSHV VLSTLWEPHI TQRQVEKAWA 
           560 
    LLQKRIPKTH QALD
    Length:564
    Mass (Da):62,329
    Last modified:June 27, 2003 - v4
    <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:i7DC9E93B356C5DB7
    GO
    Isoform 2 (identifier: Q13057-2) [UniParc]FASTAAdd to basket
    Also known as: CoASy beta

    The sequence of this isoform differs from the canonical sequence as follows:
         1-1: M → MRTPRLRAQPRGAVYQAPSPPPAPVGLGSM

    Show »
    Length:593
    Mass (Da):65,339
    Checksum:iBE77FF6197170615
    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 7 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
    EntryEntry nameProtein names
    		Gene namesLengthAnnotation
    K7EQ60K7EQ60_HUMAN
    Bifunctional coenzyme A synthase
    COASY
    		115Annotation 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>
    K7EN91K7EN91_HUMAN
    Bifunctional coenzyme A synthase
    COASY
    		219Annotation 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>
    K7ES73K7ES73_HUMAN
    Bifunctional coenzyme A synthase
    COASY
    		147Annotation 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>
    K7EP09K7EP09_HUMAN
    Bifunctional coenzyme A synthase
    COASY
    		90Annotation 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>
    K7EPC2K7EPC2_HUMAN
    Bifunctional coenzyme A synthase
    COASY
    		96Annotation 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>
    K7EPT0K7EPT0_HUMAN
    Bifunctional coenzyme A synthase
    COASY
    		98Annotation 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>
    K7ESK6K7ESK6_HUMAN
    Bifunctional coenzyme A synthase
    COASY
    		128Annotation 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>

    <p>This subsection of the 'Sequence' section reports difference(s) between the protein sequence shown in the UniProtKB entry and other available protein sequences derived from the same gene.<p><a href='/help/sequence_caution' target='_top'>More...</a></p>Sequence cautioni

    The sequence AAA69699 differs from that shown. Reason: Frameshift.Curated
    The sequence AAF87955 differs from that shown. Reason: Erroneous initiation. Truncated N-terminus.Curated
    The sequence AAH06354 differs from that shown. Reason: Erroneous initiation. Truncated N-terminus.Curated
    The sequence AAH20985 differs from that shown. Reason: Erroneous initiation. Truncated N-terminus.Curated
    The sequence AK075415 differs from that shown. Reason: Frameshift.Curated

    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 conflicti41L → P in BAG36775 (PubMed:14702039).Curated1

    Natural variant

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Natural variantiVAR_03029955S → Y4 PublicationsCorresponds to variant dbSNP:rs615942EnsemblClinVar.1
    Natural variantiVAR_08222259 – 564Missing in NBIA6; reduced protein abundance. 1 PublicationAdd BLAST506
    Natural variantiVAR_082223214A → V in NBIA6; unknown pathological significance. 1 Publication1
    Natural variantiVAR_070975499R → C in NBIA6; reduced protein abundance; loss of dephospho-CoA kinase activity. 2 PublicationsCorresponds to variant dbSNP:rs140709867EnsemblClinVar.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_0364041M → MRTPRLRAQPRGAVYQAPSP PPAPVGLGSM in isoform 2. 1 Publication1

    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
    		AF453478 mRNA Translation: AAL50813.1
    AY094602 mRNA Translation: AAM19996.1
    AK075415 mRNA No translation available.
    AK297153 mRNA Translation: BAG59652.1
    AK314076 mRNA Translation: BAG36775.1
    AC067852 Genomic DNA No translation available.
    CH471152 Genomic DNA Translation: EAW60840.1
    BC006354 mRNA Translation: AAH06354.1 Different initiation.
    BC020985 mRNA Translation: AAH20985.1 Different initiation.
    BC067254 mRNA Translation: AAH67254.1
    AF208536 mRNA Translation: AAF87955.1 Different initiation.
    U18919 mRNA Translation: AAA69699.1 Frameshift.
    BT007168 mRNA Translation: AAP35832.1

    The Consensus CDS (CCDS) project

    More...    CCDSi    		CCDS11429.1 [Q13057-1]
    CCDS45685.1 [Q13057-2]

    NCBI Reference Sequences

    More...    RefSeqi    		NP_001035994.1, NM_001042529.2 [Q13057-1]
    NP_001035997.2, NM_001042532.3 [Q13057-2]
    NP_079509.5, NM_025233.6 [Q13057-1]
    XP_006722179.1, XM_006722116.3 [Q13057-2]
    XP_011523602.1, XM_011525300.1 [Q13057-1]
    XP_016880657.1, XM_017025168.1

    Genome annotation databases

    Ensembl eukaryotic genome annotation project

    More...    Ensembli    		ENST00000393818; ENSP00000377406; ENSG00000068120 [Q13057-1]
    ENST00000421097; ENSP00000393564; ENSG00000068120 [Q13057-1]
    ENST00000590958; ENSP00000464814; ENSG00000068120 [Q13057-2]

    Database of genes from NCBI RefSeq genomes

    More...    GeneIDi    		80347

    KEGG: Kyoto Encyclopedia of Genes and Genomes

    More...    KEGGi    		hsa:80347

    UCSC genome browser

    More...    UCSCi    		uc002hzz.5, human [Q13057-1]

    Keywords - Coding sequence diversityi

    Alternative splicing, 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
    		AF453478 mRNA Translation: AAL50813.1
    AY094602 mRNA Translation: AAM19996.1
    AK075415 mRNA No translation available.
    AK297153 mRNA Translation: BAG59652.1
    AK314076 mRNA Translation: BAG36775.1
    AC067852 Genomic DNA No translation available.
    CH471152 Genomic DNA Translation: EAW60840.1
    BC006354 mRNA Translation: AAH06354.1 Different initiation.
    BC020985 mRNA Translation: AAH20985.1 Different initiation.
    BC067254 mRNA Translation: AAH67254.1
    AF208536 mRNA Translation: AAF87955.1 Different initiation.
    U18919 mRNA Translation: AAA69699.1 Frameshift.
    BT007168 mRNA Translation: AAP35832.1
    CCDSiCCDS11429.1 [Q13057-1]
    CCDS45685.1 [Q13057-2]
    RefSeqiNP_001035994.1, NM_001042529.2 [Q13057-1]
    NP_001035997.2, NM_001042532.3 [Q13057-2]
    NP_079509.5, NM_025233.6 [Q13057-1]
    XP_006722179.1, XM_006722116.3 [Q13057-2]
    XP_011523602.1, XM_011525300.1 [Q13057-1]
    XP_016880657.1, XM_017025168.1

    3D structure databases

    Database of comparative protein structure models

    More...    ModBasei    		Search...

    SWISS-MODEL Interactive Workspace

    More...    SWISS-MODEL-Workspacei    		Submit a new modelling project...

    Protein-protein interaction databases

    BioGRIDi123254, 46 interactors
    IntActiQ13057, 19 interactors
    MINTiQ13057
    STRINGi9606.ENSP00000464814

    Chemistry databases

    BindingDBiQ13057
    ChEMBLiCHEMBL4105867

    PTM databases

    iPTMnetiQ13057
    PhosphoSitePlusiQ13057

    Polymorphism and mutation databases

    BioMutaiCOASY
    DMDMi32363505

    Proteomic databases

    EPDiQ13057
    jPOSTiQ13057
    MassIVEiQ13057
    MaxQBiQ13057
    PaxDbiQ13057
    PeptideAtlasiQ13057
    PRIDEiQ13057
    ProteomicsDBi59124 [Q13057-1]
    59125 [Q13057-2]

    Protocols and materials databases

    Antibodypedia a portal for validated antibodies

    More...    Antibodypediai    		16973, 297 antibodies

    The DNASU plasmid repository

    More...    DNASUi    		80347

    Genome annotation databases

    EnsembliENST00000393818; ENSP00000377406; ENSG00000068120 [Q13057-1]
    ENST00000421097; ENSP00000393564; ENSG00000068120 [Q13057-1]
    ENST00000590958; ENSP00000464814; ENSG00000068120 [Q13057-2]
    GeneIDi80347
    KEGGihsa:80347
    UCSCiuc002hzz.5, human [Q13057-1]

    Organism-specific databases

    Comparative Toxicogenomics Database

    More...    CTDi    		80347
    DisGeNETi80347
    EuPathDBiHostDB:ENSG00000068120.14

    GeneCards: human genes, protein and diseases

    More...    GeneCardsi    		COASY
    HGNCiHGNC:29932, COASY
    HPAiENSG00000068120, Low tissue specificity
    MalaCardsiCOASY
    MIMi609855, gene
    615643, phenotype
    618266, phenotype
    neXtProtiNX_Q13057
    OpenTargetsiENSG00000068120
    Orphaneti397725, COASY protein-associated neurodegeneration
    PharmGKBiPA134867942

    GenAtlas: human gene database

    More...    GenAtlasi    		Search...

    Phylogenomic databases

    eggNOGiKOG3220, Eukaryota
    KOG3351, Eukaryota
    GeneTreeiENSGT00550000075078
    HOGENOMiCLU_027827_1_0_1
    InParanoidiQ13057
    OMAiVIDCDKV
    OrthoDBi1543581at2759
    PhylomeDBiQ13057

    Enzyme and pathway databases

    UniPathwayiUPA00241;UER00355
    UPA00241;UER00356
    BioCyciMetaCyc:HS00931-MONOMER
    BRENDAi2.7.1.24, 2681
    2.7.7.3, 2681
    PathwayCommonsiQ13057
    ReactomeiR-HSA-196783, Coenzyme A biosynthesis
    SABIO-RKiQ13057

    Miscellaneous databases

    BioGRID ORCS database of CRISPR phenotype screens

    More...    BioGRID-ORCSi    		80347, 383 hits in 853 CRISPR screens

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

    More...    ChiTaRSi    		COASY, human

    The Gene Wiki collection of pages on human genes and proteins

    More...    GeneWikii    		COASY

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

    More...    GenomeRNAii    		80347
    PharosiQ13057, Tbio

    Protein Ontology

    More...    PROi    		PR:Q13057
    RNActiQ13057, protein

    The Stanford Online Universal Resource for Clones and ESTs

    More...    SOURCEi    		Search...

    Gene expression databases

    BgeeiENSG00000068120, Expressed in body of stomach and 229 other tissues
    ExpressionAtlasiQ13057, baseline and differential
    GenevisibleiQ13057, HS

    Family and domain databases

    Gene3Di3.40.50.620, 1 hit
    HAMAPiMF_00376, Dephospho_CoA_kinase, 1 hit
    InterProiView protein in InterPro
    IPR004821, Cyt_trans-like
    IPR001977, Depp_CoAkinase
    IPR027417, P-loop_NTPase
    IPR014729, Rossmann-like_a/b/a_fold
    PfamiView protein in Pfam
    PF01121, CoaE, 1 hit
    PF01467, CTP_transf_like, 1 hit
    SUPFAMiSSF52540, SSF52540, 1 hit
    TIGRFAMsiTIGR00152, TIGR00152, 1 hit
    PROSITEiView protein in PROSITE
    PS51219, DPCK, 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 nameiCOASY_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: Q13057
    Secondary accession number(s): B2RA78
    , B4DLU0, Q6GS23, Q8NBM7, Q8NEW1, Q8WXD4, Q9NRM3
    <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: November 1, 1997
    Last sequence update: June 27, 2003
    Last modified: December 2, 2020
    This is version 200 of the entry and version 4 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 polymorphisms and disease mutations
      Index of human polymorphisms and disease mutations
    2. MIM cross-references
      Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
    3. PATHWAY comments
      Index of metabolic and biosynthesis pathways
    4. SIMILARITY comments
      Index of protein domains and families
    5. Human chromosome 17
      Human chromosome 17: entries, gene names and cross-references to MIM
    6. Human entries with polymorphisms or disease mutations
      List of human entries with polymorphisms or disease mutations
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