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Protein

Phosphopantothenoylcysteine decarboxylase

Gene

PPCDC

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Necessary for the biosynthesis of coenzyme A. Catalyzes the decarboxylation of 4-phosphopantothenoylcysteine to form 4'-phosphopantotheine.2 Publications

Catalytic activityi

N-((R)-4'-phosphopantothenoyl)-L-cysteine = pantotheine 4'-phosphate + CO2.

Cofactori

FMNNote: Binds 1 FMN per subunit.

Pathwayi: coenzyme A biosynthesis

This protein is involved in step 3 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 4 (PANK4), Pantothenate kinase 3 (PANK3), Pantothenate kinase 1 (PANK1), 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.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei59FMN1 Publication1
Binding sitei140SubstrateBy similarity1
Active sitei173Proton donor1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi104 – 107FMN1 Publication4

GO - Molecular functioni

  • identical protein binding Source: IntAct
  • phosphopantothenoylcysteine decarboxylase activity Source: UniProtKB

GO - Biological processi

  • coenzyme A biosynthetic process Source: UniProtKB
  • coenzyme biosynthetic process Source: Reactome
Complete GO annotation...

Keywords - Molecular functioni

Decarboxylase, Lyase

Keywords - Biological processi

Coenzyme A biosynthesis

Keywords - Ligandi

Flavoprotein, FMN

Enzyme and pathway databases

BioCyciMetaCyc:HS13735-MONOMER.
ZFISH:ENSG00000103187-MONOMER.
ZFISH:HS13735-MONOMER.
ReactomeiR-HSA-196783. Coenzyme A biosynthesis.
SABIO-RKQ96CD2.
UniPathwayiUPA00241; UER00354.

Names & Taxonomyi

Protein namesi
Recommended name:
Phosphopantothenoylcysteine decarboxylase (EC:4.1.1.36)
Short name:
PPC-DC
Alternative name(s):
CoaC
Gene namesi
Name:PPCDC
Synonyms:COAC
ORF Names:MDS018, UNQ9365/PRO34154
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 15

Organism-specific databases

HGNCiHGNC:28107. PPCDC.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

Pathology & Biotechi

Organism-specific databases

DisGeNETi60490.
OpenTargetsiENSG00000138621.
PharmGKBiPA142671157.

Polymorphism and mutation databases

BioMutaiPPCDC.
DMDMi296434457.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00001820301 – 204Phosphopantothenoylcysteine decarboxylaseAdd BLAST204

Proteomic databases

EPDiQ96CD2.
MaxQBiQ96CD2.
PaxDbiQ96CD2.
PeptideAtlasiQ96CD2.
PRIDEiQ96CD2.

PTM databases

iPTMnetiQ96CD2.
PhosphoSitePlusiQ96CD2.

Expressioni

Gene expression databases

BgeeiENSG00000138621.
CleanExiHS_PPCDC.
ExpressionAtlasiQ96CD2. baseline and differential.
GenevisibleiQ96CD2. HS.

Organism-specific databases

HPAiHPA045667.

Interactioni

Subunit structurei

Homotrimer.Curated

Binary interactionsi

WithEntry#Exp.IntActNotes
itself4EBI-724333,EBI-724333
FOXR1Q6PIV23EBI-724333,EBI-10253815
PPIGQ134273EBI-724333,EBI-396072
SREK1IP1Q8N9Q23EBI-724333,EBI-10268630
TXN2Q997575EBI-724333,EBI-2932492
WDYHV1Q96HA85EBI-724333,EBI-741158
ZNF232Q9UNY55EBI-724333,EBI-749023

GO - Molecular functioni

  • identical protein binding Source: IntAct

Protein-protein interaction databases

BioGridi121921. 8 interactors.
IntActiQ96CD2. 13 interactors.
MINTiMINT-1394867.
STRINGi9606.ENSP00000343190.

Structurei

Secondary structure

1204
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi16 – 24Combined sources9
Helixi28 – 31Combined sources4
Helixi33 – 40Combined sources8
Beta strandi46 – 52Combined sources7
Helixi55 – 58Combined sources4
Helixi62 – 64Combined sources3
Helixi72 – 77Combined sources6
Helixi87 – 92Combined sources6
Beta strandi96 – 103Combined sources8
Helixi105 – 112Combined sources8
Helixi119 – 125Combined sources7
Beta strandi135 – 137Combined sources3
Helixi141 – 144Combined sources4
Helixi149 – 157Combined sources9
Helixi186 – 191Combined sources6

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1QZUX-ray2.91A/B/C/D1-204[»]
ProteinModelPortaliQ96CD2.
SMRiQ96CD2.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiQ96CD2.

Family & Domainsi

Sequence similaritiesi

Phylogenomic databases

eggNOGiKOG0672. Eukaryota.
COG0452. LUCA.
GeneTreeiENSGT00440000038107.
HOGENOMiHOG000187629.
HOVERGENiHBG054722.
InParanoidiQ96CD2.
KOiK01598.
OMAiKHFYSPQ.
OrthoDBiEOG091G0SB2.
PhylomeDBiQ96CD2.
TreeFamiTF315740.

Family and domain databases

Gene3Di3.40.50.1950. 1 hit.
InterProiIPR003382. Flavoprotein.
[Graphical view]
PfamiPF02441. Flavoprotein. 1 hit.
[Graphical view]
SUPFAMiSSF52507. SSF52507. 1 hit.

Sequences (2)i

Sequence statusi: Complete.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q96CD2-1) [UniParc]FASTAAdd to basket

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
MEPKASCPAA APLMERKFHV LVGVTGSVAA LKLPLLVSKL LDIPGLEVAV
60 70 80 90 100
VTTERAKHFY SPQDIPVTLY SDADEWEIWK SRSDPVLHID LRRWADLLLV
110 120 130 140 150
APLDANTLGK VASGICDNLL TCVMRAWDRS KPLLFCPAMN TAMWEHPITA
160 170 180 190 200
QQVDQLKAFG YVEIPCVAKK LVCGDEGLGA MAEVGTIVDK VKEVLFQHSG

FQQS
Length:204
Mass (Da):22,395
Last modified:May 18, 2010 - v2
Checksum:i9E59683480032894
GO
Isoform 2 (identifier: Q96CD2-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-78: MEPKASCPAA...LYSDADEWEI → M

Note: The Met-1 codon is associated with a polymorphism (dbSNP:rs2304899) that replaces the initiation ATG codon by an ATA codon.
Show »
Length:127
Mass (Da):14,069
Checksum:i562D659DE77C0AF5
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti49A → S in BAB55151 (PubMed:14702039).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_06897478I → M.Corresponds to variant rs2304899dbSNPEnsembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0448021 – 78MEPKA…DEWEI → M in isoform 2. 1 PublicationAdd BLAST78

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF182419 mRNA. Translation: AAG14955.1.
AY358848 mRNA. Translation: AAQ89207.1.
AK027491 mRNA. Translation: BAB55151.1.
AC015720 Genomic DNA. No translation available.
BC014409 mRNA. Translation: AAH14409.1.
CCDSiCCDS10275.1. [Q96CD2-1]
RefSeqiNP_001288030.1. NM_001301101.1.
NP_001288031.1. NM_001301102.1.
NP_001288032.1. NM_001301103.1.
NP_001288033.1. NM_001301104.1.
NP_001288034.1. NM_001301105.1.
NP_068595.3. NM_021823.4. [Q96CD2-1]
UniGeneiHs.458922.

Genome annotation databases

EnsembliENST00000342932; ENSP00000343190; ENSG00000138621. [Q96CD2-1]
GeneIDi60490.
KEGGihsa:60490.
UCSCiuc002azo.4. human. [Q96CD2-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF182419 mRNA. Translation: AAG14955.1.
AY358848 mRNA. Translation: AAQ89207.1.
AK027491 mRNA. Translation: BAB55151.1.
AC015720 Genomic DNA. No translation available.
BC014409 mRNA. Translation: AAH14409.1.
CCDSiCCDS10275.1. [Q96CD2-1]
RefSeqiNP_001288030.1. NM_001301101.1.
NP_001288031.1. NM_001301102.1.
NP_001288032.1. NM_001301103.1.
NP_001288033.1. NM_001301104.1.
NP_001288034.1. NM_001301105.1.
NP_068595.3. NM_021823.4. [Q96CD2-1]
UniGeneiHs.458922.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1QZUX-ray2.91A/B/C/D1-204[»]
ProteinModelPortaliQ96CD2.
SMRiQ96CD2.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi121921. 8 interactors.
IntActiQ96CD2. 13 interactors.
MINTiMINT-1394867.
STRINGi9606.ENSP00000343190.

PTM databases

iPTMnetiQ96CD2.
PhosphoSitePlusiQ96CD2.

Polymorphism and mutation databases

BioMutaiPPCDC.
DMDMi296434457.

Proteomic databases

EPDiQ96CD2.
MaxQBiQ96CD2.
PaxDbiQ96CD2.
PeptideAtlasiQ96CD2.
PRIDEiQ96CD2.

Protocols and materials databases

DNASUi60490.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000342932; ENSP00000343190; ENSG00000138621. [Q96CD2-1]
GeneIDi60490.
KEGGihsa:60490.
UCSCiuc002azo.4. human. [Q96CD2-1]

Organism-specific databases

CTDi60490.
DisGeNETi60490.
GeneCardsiPPCDC.
H-InvDBHIX0202140.
HGNCiHGNC:28107. PPCDC.
HPAiHPA045667.
MIMi609854. gene.
neXtProtiNX_Q96CD2.
OpenTargetsiENSG00000138621.
PharmGKBiPA142671157.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG0672. Eukaryota.
COG0452. LUCA.
GeneTreeiENSGT00440000038107.
HOGENOMiHOG000187629.
HOVERGENiHBG054722.
InParanoidiQ96CD2.
KOiK01598.
OMAiKHFYSPQ.
OrthoDBiEOG091G0SB2.
PhylomeDBiQ96CD2.
TreeFamiTF315740.

Enzyme and pathway databases

UniPathwayiUPA00241; UER00354.
BioCyciMetaCyc:HS13735-MONOMER.
ZFISH:ENSG00000103187-MONOMER.
ZFISH:HS13735-MONOMER.
ReactomeiR-HSA-196783. Coenzyme A biosynthesis.
SABIO-RKQ96CD2.

Miscellaneous databases

EvolutionaryTraceiQ96CD2.
GenomeRNAii60490.
PROiQ96CD2.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000138621.
CleanExiHS_PPCDC.
ExpressionAtlasiQ96CD2. baseline and differential.
GenevisibleiQ96CD2. HS.

Family and domain databases

Gene3Di3.40.50.1950. 1 hit.
InterProiIPR003382. Flavoprotein.
[Graphical view]
PfamiPF02441. Flavoprotein. 1 hit.
[Graphical view]
SUPFAMiSSF52507. SSF52507. 1 hit.
ProtoNetiSearch...

Entry informationi

Entry nameiCOAC_HUMAN
AccessioniPrimary (citable) accession number: Q96CD2
Secondary accession number(s): Q96SX0, Q9HC17
Entry historyi
Integrated into UniProtKB/Swiss-Prot: June 7, 2005
Last sequence update: May 18, 2010
Last modified: November 30, 2016
This is version 128 of the entry and version 2 of the sequence. [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

3D-structure, Complete proteome, Reference proteome

Documents

  1. Human chromosome 15
    Human chromosome 15: entries, gene names and cross-references to MIM
  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. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  7. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.