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Protein

Bifunctional coenzyme A synthase

Gene

COASY

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

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.1 Publication

Catalytic activityi

ATP + pantetheine 4'-phosphate = diphosphate + 3'-dephospho-CoA.
ATP + 3'-dephospho-CoA = ADP + CoA.

Kineticsi

  1. KM=7.6 mM for 4'-phoshopantetheine
  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)

    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 4 (PANK4), Pantothenate kinase 2, mitochondrial (PANK2), Pantothenate kinase 4 (PANK4), Pantothenate kinase 4, Pantothenate kinase 4
    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
    Nucleotide bindingi365 – 372ATPSequence analysis8

    GO - Molecular functioni

    GO - Biological processi

    Keywordsi

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

    Enzyme and pathway databases

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

    UPA00241;UER00356

    Names & Taxonomyi

    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:2.7.1.24)
    Short name:
    DPCK
    Alternative name(s):
    Dephosphocoenzyme A kinase
    Short name:
    DPCOAK
    Gene namesi
    Name:COASY
    ORF Names:PSEC0106
    OrganismiHomo sapiens (Human)
    Taxonomic identifieri9606 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    Proteomesi
    • UP000005640 Componenti: Chromosome 17

    Organism-specific databases

    EuPathDBiHostDB:ENSG00000068120.14
    HGNCiHGNC:29932 COASY
    MIMi609855 gene
    neXtProtiNX_Q13057

    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; Source: COMPARTMENTS

    Keywords - Cellular componenti

    Cytoplasm, Mitochondrion

    Pathology & Biotechi

    Involvement in diseasei

    Neurodegeneration with brain iron accumulation 6 (NBIA6)1 Publication
    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.
    See also OMIM:615643
    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Natural variantiVAR_070975499R → C in NBIA6. 1 PublicationCorresponds to variant dbSNP:rs140709867EnsemblClinVar.1

    Keywords - Diseasei

    Disease mutation, Neurodegeneration

    Organism-specific databases

    DisGeNETi80347
    MalaCardsiCOASY
    MIMi615643 phenotype
    OpenTargetsiENSG00000068120
    Orphaneti397725 COASY protein-associated neurodegeneration
    PharmGKBiPA134867942

    Polymorphism and mutation databases

    BioMutaiCOASY
    DMDMi32363505

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    ChainiPRO_00001730391 – 564Bifunctional coenzyme A synthaseAdd BLAST564

    Amino acid modifications

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Modified residuei178PhosphoserineCombined sources1
    Modified residuei183PhosphoserineCombined sources1

    Keywords - PTMi

    Phosphoprotein

    Proteomic databases

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

    PTM databases

    iPTMnetiQ13057
    PhosphoSitePlusiQ13057

    Expressioni

    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

    BgeeiENSG00000068120 Expressed in 216 organ(s), highest expression level in body of stomach
    CleanExiHS_COASY
    ExpressionAtlasiQ13057 baseline and differential
    GenevisibleiQ13057 HS

    Organism-specific databases

    HPAiHPA022875
    HPA022912
    HPA023273

    Interactioni

    Subunit structurei

    Monomer.1 Publication

    Binary interactionsi

    Protein-protein interaction databases

    BioGridi123254, 29 interactors
    IntActiQ13057, 8 interactors
    MINTiQ13057
    STRINGi9606.ENSP00000464814

    Structurei

    3D structure databases

    ProteinModelPortaliQ13057
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Domains and Repeats

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Domaini360 – 563DPCKAdd BLAST204

    Region

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Regioni180 – 358Phosphopantetheine adenylyltransferaseAdd BLAST179

    Sequence similaritiesi

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

    Phylogenomic databases

    eggNOGiKOG3220 Eukaryota
    KOG3351 Eukaryota
    COG0237 LUCA
    COG1019 LUCA
    GeneTreeiENSGT00550000075078
    HOVERGENiHBG051059
    InParanoidiQ13057
    KOiK02318
    OMAiVFGNKKQ
    OrthoDBiEOG091G053D
    PhylomeDBiQ13057

    Family and domain databases

    CDDicd02022 DPCK, 1 hit
    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

    Sequences (2+)i

    Sequence statusi: Complete.

    This entry describes 2 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 'canonical' 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
    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

    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:
    K7EN91K7EN91_HUMAN
    Bifunctional coenzyme A synthase
    COASY
    219Annotation score:
    K7ES73K7ES73_HUMAN
    Bifunctional coenzyme A synthase
    COASY
    147Annotation score:
    K7EP09K7EP09_HUMAN
    Bifunctional coenzyme A synthase
    COASY
    90Annotation score:
    K7EPC2K7EPC2_HUMAN
    Bifunctional coenzyme A synthase
    COASY
    96Annotation score:
    K7EPT0K7EPT0_HUMAN
    Bifunctional coenzyme A synthase
    COASY
    98Annotation score:
    K7ESK6K7ESK6_HUMAN
    Bifunctional coenzyme A synthase
    COASY
    128Annotation score:

    Sequence cautioni

    The sequence AAA69699 differs from that shown. Reason: Frameshift at position 535.Curated
    The sequence AAF87955 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated
    The sequence AAH06354 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated
    The sequence AAH20985 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated
    The sequence AK075415 differs from that shown. Reason: Frameshift at position 315.Curated

    Experimental Info

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    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_070975499R → C in NBIA6. 1 PublicationCorresponds to variant dbSNP:rs140709867EnsemblClinVar.1

    Alternative sequence

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Alternative sequenceiVSP_0364041M → MRTPRLRAQPRGAVYQAPSP PPAPVGLGSM in isoform 2. 1 Publication1

    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
    UniGeneiHs.296422
    Hs.742262

    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]

    Keywords - Coding sequence diversityi

    Alternative splicing, Polymorphism

    Similar proteinsi

    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
    UniGeneiHs.296422
    Hs.742262

    3D structure databases

    ProteinModelPortaliQ13057
    ModBaseiSearch...
    MobiDBiSearch...

    Protein-protein interaction databases

    BioGridi123254, 29 interactors
    IntActiQ13057, 8 interactors
    MINTiQ13057
    STRINGi9606.ENSP00000464814

    PTM databases

    iPTMnetiQ13057
    PhosphoSitePlusiQ13057

    Polymorphism and mutation databases

    BioMutaiCOASY
    DMDMi32363505

    Proteomic databases

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

    Protocols and materials databases

    DNASUi80347
    Structural Biology KnowledgebaseSearch...

    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

    CTDi80347
    DisGeNETi80347
    EuPathDBiHostDB:ENSG00000068120.14
    GeneCardsiCOASY
    HGNCiHGNC:29932 COASY
    HPAiHPA022875
    HPA022912
    HPA023273
    MalaCardsiCOASY
    MIMi609855 gene
    615643 phenotype
    neXtProtiNX_Q13057
    OpenTargetsiENSG00000068120
    Orphaneti397725 COASY protein-associated neurodegeneration
    PharmGKBiPA134867942
    GenAtlasiSearch...

    Phylogenomic databases

    eggNOGiKOG3220 Eukaryota
    KOG3351 Eukaryota
    COG0237 LUCA
    COG1019 LUCA
    GeneTreeiENSGT00550000075078
    HOVERGENiHBG051059
    InParanoidiQ13057
    KOiK02318
    OMAiVFGNKKQ
    OrthoDBiEOG091G053D
    PhylomeDBiQ13057

    Enzyme and pathway databases

    UniPathwayi
    UPA00241;UER00355

    UPA00241;UER00356

    BioCyciMetaCyc:HS00931-MONOMER
    BRENDAi2.7.1.24 2681
    2.7.7.3 2681
    ReactomeiR-HSA-196783 Coenzyme A biosynthesis
    SABIO-RKiQ13057

    Miscellaneous databases

    ChiTaRSiCOASY human
    GeneWikiiCOASY
    GenomeRNAii80347
    PROiPR:Q13057
    SOURCEiSearch...

    Gene expression databases

    BgeeiENSG00000068120 Expressed in 216 organ(s), highest expression level in body of stomach
    CleanExiHS_COASY
    ExpressionAtlasiQ13057 baseline and differential
    GenevisibleiQ13057 HS

    Family and domain databases

    CDDicd02022 DPCK, 1 hit
    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
    ProtoNetiSearch...

    Entry informationi

    Entry nameiCOASY_HUMAN
    AccessioniPrimary (citable) accession number: Q13057
    Secondary accession number(s): B2RA78
    , B4DLU0, Q6GS23, Q8NBM7, Q8NEW1, Q8WXD4, Q9NRM3
    Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 1, 1997
    Last sequence update: June 27, 2003
    Last modified: November 7, 2018
    This is version 183 of the entry and version 4 of the sequence. See complete history.
    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.

    Miscellaneousi

    Keywords - Technical termi

    Complete proteome, Reference proteome

    Documents

    1. SIMILARITY comments
      Index of protein domains and families
    2. Human entries with polymorphisms or disease mutations
      List of human entries with polymorphisms or disease mutations
    3. Human polymorphisms and disease mutations
      Index of human polymorphisms and disease mutations
    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. Human chromosome 17
      Human chromosome 17: entries, gene names and cross-references to MIM
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