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Protein

Phosphopantothenate--cysteine ligase

Gene

PPCS

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

Functioni

Catalyzes the first step in the biosynthesis of coenzyme A from vitamin B5, where cysteine is conjugated to 4'-phosphopantothenate to form 4-phosphopantothenoylcysteine.2 Publications

Catalytic activityi

CTP + (R)-4'-phosphopantothenate + L-cysteine = CMP + diphosphate + N-((R)-4'-phosphopantothenoyl)-L-cysteine.

Pathwayi: coenzyme A biosynthesis

This protein is involved in step 2 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.

GO - Molecular functioni

  • phosphopantothenate--cysteine ligase activity Source: UniProtKB

GO - Biological processi

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

Keywords - Molecular functioni

Ligase

Enzyme and pathway databases

BioCyciMetaCyc:HS13229-MONOMER.
ZFISH:HS13229-MONOMER.
ReactomeiR-HSA-196783. Coenzyme A biosynthesis.
UniPathwayiUPA00241; UER00353.

Names & Taxonomyi

Protein namesi
Recommended name:
Phosphopantothenate--cysteine ligase (EC:6.3.2.5)
Alternative name(s):
Phosphopantothenoylcysteine synthetase
Short name:
PPC synthetase
Gene namesi
Name:PPCS
Synonyms:COAB
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 1

Organism-specific databases

HGNCiHGNC:25686. PPCS.

Subcellular locationi

GO - Cellular componenti

  • cytosol Source: Reactome
  • extracellular exosome Source: UniProtKB
Complete GO annotation...

Pathology & Biotechi

Organism-specific databases

OpenTargetsiENSG00000127125.
PharmGKBiPA142671158.

Polymorphism and mutation databases

DMDMi47117318.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemovedCombined sources
ChainiPRO_00001820402 – 311Phosphopantothenate--cysteine ligaseAdd BLAST310

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei2N-acetylalanineCombined sources1

Keywords - PTMi

Acetylation

Proteomic databases

EPDiQ9HAB8.
MaxQBiQ9HAB8.
PaxDbiQ9HAB8.
PeptideAtlasiQ9HAB8.
PRIDEiQ9HAB8.
TopDownProteomicsiQ9HAB8-1. [Q9HAB8-1]

PTM databases

iPTMnetiQ9HAB8.
PhosphoSitePlusiQ9HAB8.

Expressioni

Gene expression databases

BgeeiENSG00000127125.
CleanExiHS_PPCS.
ExpressionAtlasiQ9HAB8. baseline and differential.
GenevisibleiQ9HAB8. HS.

Organism-specific databases

HPAiHPA031361.
HPA031363.

Interactioni

Subunit structurei

Homodimer.

Protein-protein interaction databases

BioGridi122833. 3 interactors.
IntActiQ9HAB8. 1 interactor.
STRINGi9606.ENSP00000361642.

Structurei

Secondary structure

1311
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Helixi19 – 32Combined sources14
Beta strandi37 – 44Combined sources8
Beta strandi46 – 52Combined sources7
Beta strandi54 – 59Combined sources6
Helixi64 – 75Combined sources12
Beta strandi79 – 85Combined sources7
Helixi93 – 95Combined sources3
Helixi98 – 104Combined sources7
Beta strandi114 – 121Combined sources8
Turni122 – 124Combined sources3
Helixi128 – 141Combined sources14
Beta strandi144 – 149Combined sources6
Helixi152 – 166Combined sources15
Helixi167 – 172Combined sources6
Beta strandi173 – 177Combined sources5
Beta strandi183 – 185Combined sources3
Beta strandi203 – 206Combined sources4
Helixi211 – 214Combined sources4
Helixi218 – 220Combined sources3
Beta strandi225 – 231Combined sources7
Helixi236 – 250Combined sources15
Beta strandi253 – 258Combined sources6
Beta strandi267 – 271Combined sources5
Beta strandi274 – 278Combined sources5
Helixi282 – 286Combined sources5
Helixi291 – 306Combined sources16

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1P9OX-ray2.30A/B1-311[»]
ProteinModelPortaliQ9HAB8.
SMRiQ9HAB8.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiQ9HAB8.

Family & Domainsi

Sequence similaritiesi

Belongs to the PPC synthetase family.Curated

Phylogenomic databases

eggNOGiKOG2728. Eukaryota.
COG0452. LUCA.
GeneTreeiENSGT00390000015263.
HOGENOMiHOG000194726.
HOVERGENiHBG049438.
InParanoidiQ9HAB8.
KOiK01922.
OMAiDPVAEFP.
OrthoDBiEOG091G0R3S.
PhylomeDBiQ9HAB8.
TreeFamiTF105615.

Family and domain databases

Gene3Di3.40.50.10300. 1 hit.
InterProiIPR007085. DNA/pantothenate-metab_flavo_C.
[Graphical view]
PfamiPF04127. DFP. 2 hits.
[Graphical view]
SUPFAMiSSF102645. SSF102645. 1 hit.

Sequences (2)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

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

Isoform 1 (identifier: Q9HAB8-1) [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
MAEMDPVAEF PQPPGAARWA EVMARFAARL GAQGRRVVLV TSGGTKVPLE
60 70 80 90 100
ARPVRFLDNF SSGRRGATSA EAFLAAGYGV LFLYRARSAF PYAHRFPPQT
110 120 130 140 150
WLSALRPSGP ALSGLLSLEA EENALPGFAE ALRSYQEAAA AGTFLAVEFT
160 170 180 190 200
TLADYLHLLQ AAAQALNPLG PSAMFYLAAA VSDFYVPVSE MPEHKIQSSG
210 220 230 240 250
GPLQITMKMV PKLLSPLVKD WAPKAFIISF KLETDPAIVI NRARKALEIY
260 270 280 290 300
QHQVVVANIL ESRQSFVFIV TKDSETKLLL SEEEIEKGVE IEEKIVDNLQ
310
SRHTAFIGDR N
Length:311
Mass (Da):34,005
Last modified:May 10, 2004 - v2
Checksum:iD3B337D3250D7170
GO
Isoform 2 (identifier: Q9HAB8-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-173: Missing.

Note: No experimental confirmation available.
Show »
Length:138
Mass (Da):15,645
Checksum:i67B9F972DCC2B60F
GO

Sequence cautioni

The sequence BAB13931 differs from that shown. Reason: Frameshift at position 282.Curated

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0457961 – 173Missing in isoform 2. 1 PublicationAdd BLAST173

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK021900 mRNA. Translation: BAB13931.1. Frameshift.
AL445669 Genomic DNA. Translation: CAH70748.1.
AL445669 Genomic DNA. Translation: CAH70749.1.
BC012383 mRNA. No translation available.
BC104938 mRNA. Translation: AAI04939.1.
BC106064 mRNA. Translation: AAI06065.1.
BC112015 mRNA. Translation: AAI12016.1.
CCDSiCCDS41311.1. [Q9HAB8-1]
CCDS41312.1. [Q9HAB8-2]
RefSeqiNP_001070915.1. NM_001077447.2. [Q9HAB8-2]
NP_001274435.1. NM_001287506.1. [Q9HAB8-2]
NP_001274437.1. NM_001287508.1. [Q9HAB8-2]
NP_001274438.1. NM_001287509.1. [Q9HAB8-2]
NP_001274439.1. NM_001287510.1. [Q9HAB8-2]
NP_001274440.1. NM_001287511.1.
NP_078940.2. NM_024664.3. [Q9HAB8-1]
UniGeneiHs.706662.

Genome annotation databases

EnsembliENST00000372561; ENSP00000361642; ENSG00000127125. [Q9HAB8-1]
ENST00000372562; ENSP00000361643; ENSG00000127125. [Q9HAB8-2]
GeneIDi79717.
KEGGihsa:79717.
UCSCiuc001chl.5. human. [Q9HAB8-1]

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK021900 mRNA. Translation: BAB13931.1. Frameshift.
AL445669 Genomic DNA. Translation: CAH70748.1.
AL445669 Genomic DNA. Translation: CAH70749.1.
BC012383 mRNA. No translation available.
BC104938 mRNA. Translation: AAI04939.1.
BC106064 mRNA. Translation: AAI06065.1.
BC112015 mRNA. Translation: AAI12016.1.
CCDSiCCDS41311.1. [Q9HAB8-1]
CCDS41312.1. [Q9HAB8-2]
RefSeqiNP_001070915.1. NM_001077447.2. [Q9HAB8-2]
NP_001274435.1. NM_001287506.1. [Q9HAB8-2]
NP_001274437.1. NM_001287508.1. [Q9HAB8-2]
NP_001274438.1. NM_001287509.1. [Q9HAB8-2]
NP_001274439.1. NM_001287510.1. [Q9HAB8-2]
NP_001274440.1. NM_001287511.1.
NP_078940.2. NM_024664.3. [Q9HAB8-1]
UniGeneiHs.706662.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1P9OX-ray2.30A/B1-311[»]
ProteinModelPortaliQ9HAB8.
SMRiQ9HAB8.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi122833. 3 interactors.
IntActiQ9HAB8. 1 interactor.
STRINGi9606.ENSP00000361642.

PTM databases

iPTMnetiQ9HAB8.
PhosphoSitePlusiQ9HAB8.

Polymorphism and mutation databases

DMDMi47117318.

Proteomic databases

EPDiQ9HAB8.
MaxQBiQ9HAB8.
PaxDbiQ9HAB8.
PeptideAtlasiQ9HAB8.
PRIDEiQ9HAB8.
TopDownProteomicsiQ9HAB8-1. [Q9HAB8-1]

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000372561; ENSP00000361642; ENSG00000127125. [Q9HAB8-1]
ENST00000372562; ENSP00000361643; ENSG00000127125. [Q9HAB8-2]
GeneIDi79717.
KEGGihsa:79717.
UCSCiuc001chl.5. human. [Q9HAB8-1]

Organism-specific databases

CTDi79717.
GeneCardsiPPCS.
HGNCiHGNC:25686. PPCS.
HPAiHPA031361.
HPA031363.
MIMi609853. gene.
neXtProtiNX_Q9HAB8.
OpenTargetsiENSG00000127125.
PharmGKBiPA142671158.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG2728. Eukaryota.
COG0452. LUCA.
GeneTreeiENSGT00390000015263.
HOGENOMiHOG000194726.
HOVERGENiHBG049438.
InParanoidiQ9HAB8.
KOiK01922.
OMAiDPVAEFP.
OrthoDBiEOG091G0R3S.
PhylomeDBiQ9HAB8.
TreeFamiTF105615.

Enzyme and pathway databases

UniPathwayiUPA00241; UER00353.
BioCyciMetaCyc:HS13229-MONOMER.
ZFISH:HS13229-MONOMER.
ReactomeiR-HSA-196783. Coenzyme A biosynthesis.

Miscellaneous databases

EvolutionaryTraceiQ9HAB8.
GeneWikiiPhosphopantothenate%E2%80%94cysteine_ligase.
GenomeRNAii79717.
PROiQ9HAB8.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000127125.
CleanExiHS_PPCS.
ExpressionAtlasiQ9HAB8. baseline and differential.
GenevisibleiQ9HAB8. HS.

Family and domain databases

Gene3Di3.40.50.10300. 1 hit.
InterProiIPR007085. DNA/pantothenate-metab_flavo_C.
[Graphical view]
PfamiPF04127. DFP. 2 hits.
[Graphical view]
SUPFAMiSSF102645. SSF102645. 1 hit.
ProtoNetiSearch...

Entry informationi

Entry nameiPPCS_HUMAN
AccessioniPrimary (citable) accession number: Q9HAB8
Secondary accession number(s): Q3KQT2, Q5VVM0
Entry historyi
Integrated into UniProtKB/Swiss-Prot: May 10, 2004
Last sequence update: May 10, 2004
Last modified: November 2, 2016
This is version 139 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

Miscellaneous

The mammalian enzyme has a preference for ATP over CTP, in contrast to the E.coli ortholog.

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. Human chromosome 1
    Human chromosome 1: entries, gene names and cross-references to MIM
  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. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  5. 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.