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Protein

Phosphopantothenate--cysteine ligase

Gene

PPCS

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -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

Miscellaneous

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

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 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.

GO - Molecular functioni

  • phosphopantothenate--cysteine ligase activity Source: UniProtKB

GO - Biological processi

Keywordsi

Molecular functionLigase

Enzyme and pathway databases

BioCyciMetaCyc:HS13229-MONOMER
ReactomeiR-HSA-196783 Coenzyme A biosynthesis
UniPathwayi
UPA00241;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

EuPathDBiHostDB:ENSG00000127125.8
HGNCiHGNC:25686 PPCS
MIMi609853 gene
neXtProtiNX_Q9HAB8

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

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
ProteomicsDBi81390
TopDownProteomicsiQ9HAB8-1 [Q9HAB8-1]

PTM databases

iPTMnetiQ9HAB8
PhosphoSitePlusiQ9HAB8

Expressioni

Gene expression databases

BgeeiENSG00000127125 Expressed in 216 organ(s), highest expression level in body of pancreas
CleanExiHS_PPCS
ExpressionAtlasiQ9HAB8 baseline and differential
GenevisibleiQ9HAB8 HS

Organism-specific databases

HPAiHPA031361
HPA031363

Interactioni

Subunit structurei

Homodimer.

Protein-protein interaction databases

BioGridi122833, 31 interactors
IntActiQ9HAB8, 1 interactor
STRINGi9606.ENSP00000361642

Structurei

Secondary structure

1311
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

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
OMAiQPFSRHY
OrthoDBiEOG091G0R3S
PhylomeDBiQ9HAB8
TreeFamiTF105615

Family and domain databases

Gene3Di3.40.50.10300, 1 hit
InterProiView protein in InterPro
IPR035929 CoaB-like_sf
IPR007085 DNA/pantothenate-metab_flavo_C
PfamiView protein in Pfam
PF04127 DFP, 2 hits
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

This entry has 2 described isoforms and 2 potential isoforms that are computationally mapped.Show allAlign All

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

Computationally mapped potential isoform sequencesi

There are 2 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
Q5VVM3Q5VVM3_HUMAN
Phosphopantothenate--cysteine ligas...
PPCS
213Annotation score:
Q5VVM2Q5VVM2_HUMAN
Phosphopantothenate--cysteine ligas...
PPCS
56Annotation score:

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 No translation available.
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

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK021900 mRNA Translation: BAB13931.1 Frameshift.
AL445669 Genomic DNA No translation available.
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, 31 interactors
IntActiQ9HAB8, 1 interactor
STRINGi9606.ENSP00000361642

PTM databases

iPTMnetiQ9HAB8
PhosphoSitePlusiQ9HAB8

Polymorphism and mutation databases

DMDMi47117318

Proteomic databases

EPDiQ9HAB8
MaxQBiQ9HAB8
PaxDbiQ9HAB8
PeptideAtlasiQ9HAB8
PRIDEiQ9HAB8
ProteomicsDBi81390
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
EuPathDBiHostDB:ENSG00000127125.8
GeneCardsiPPCS
HGNCiHGNC:25686 PPCS
HPAiHPA031361
HPA031363
MIMi609853 gene
neXtProtiNX_Q9HAB8
OpenTargetsiENSG00000127125
PharmGKBiPA142671158
GenAtlasiSearch...

Phylogenomic databases

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

Enzyme and pathway databases

UniPathwayi
UPA00241;UER00353

BioCyciMetaCyc:HS13229-MONOMER
ReactomeiR-HSA-196783 Coenzyme A biosynthesis

Miscellaneous databases

EvolutionaryTraceiQ9HAB8
GeneWikiiPhosphopantothenate%E2%80%94cysteine_ligase
GenomeRNAii79717
PROiPR:Q9HAB8
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000127125 Expressed in 216 organ(s), highest expression level in body of pancreas
CleanExiHS_PPCS
ExpressionAtlasiQ9HAB8 baseline and differential
GenevisibleiQ9HAB8 HS

Family and domain databases

Gene3Di3.40.50.10300, 1 hit
InterProiView protein in InterPro
IPR035929 CoaB-like_sf
IPR007085 DNA/pantothenate-metab_flavo_C
PfamiView protein in Pfam
PF04127 DFP, 2 hits
SUPFAMiSSF102645 SSF102645, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiPPCS_HUMAN
AccessioniPrimary (citable) accession number: Q9HAB8
Secondary accession number(s): Q3KQT2, Q5VVM0
Entry historyiIntegrated into UniProtKB/Swiss-Prot: May 10, 2004
Last sequence update: May 10, 2004
Last modified: November 7, 2018
This is version 151 of the entry and version 2 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

3D-structure, Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. Human chromosome 1
    Human chromosome 1: entries, gene names and cross-references to MIM
  3. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  4. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
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Main funding by: National Institutes of Health

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