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Protein

CDP-diacylglycerol--glycerol-3-phosphate 3-phosphatidyltransferase, mitochondrial

Gene

PGS1

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

Functioni

Functions in the biosynthesis of the anionic phospholipids phosphatidylglycerol and cardiolipin.By similarity

Catalytic activityi

CDP-diacylglycerol + sn-glycerol 3-phosphate = CMP + 3(3-sn-phosphatidyl)-sn-glycerol 1-phosphate.

Enzyme regulationi

Activated by calcium and magnesium and inhibited by other bivalent cations.By similarity

Pathwayi: phosphatidylglycerol biosynthesis

This protein is involved in step 1 of the subpathway that synthesizes phosphatidylglycerol from CDP-diacylglycerol.
Proteins known to be involved in the 2 steps of the subpathway in this organism are:
  1. CDP-diacylglycerol--glycerol-3-phosphate 3-phosphatidyltransferase, mitochondrial (PGS1)
  2. Phosphatidylglycerophosphatase and protein-tyrosine phosphatase 1 (PTPMT1)
This subpathway is part of the pathway phosphatidylglycerol biosynthesis, which is itself part of Phospholipid metabolism.
View all proteins of this organism that are known to be involved in the subpathway that synthesizes phosphatidylglycerol from CDP-diacylglycerol, the pathway phosphatidylglycerol biosynthesis and in Phospholipid metabolism.

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Active sitei220 – 2201PROSITE-ProRule annotation
Active sitei222 – 2221PROSITE-ProRule annotation
Active sitei227 – 2271PROSITE-ProRule annotation

Regions

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Nucleotide bindingi124 – 1318ATPSequence analysis

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Transferase

Keywords - Biological processi

Lipid biosynthesis, Lipid metabolism, Phospholipid biosynthesis, Phospholipid metabolism

Keywords - Ligandi

ATP-binding, Nucleotide-binding

Enzyme and pathway databases

BioCyciMetaCyc:HS01560-MONOMER.
ReactomeiR-HSA-1483148. Synthesis of PG.
UniPathwayiUPA00084; UER00503.

Names & Taxonomyi

Protein namesi
Recommended name:
CDP-diacylglycerol--glycerol-3-phosphate 3-phosphatidyltransferase, mitochondrial (EC:2.7.8.5)
Alternative name(s):
Phosphatidylglycerophosphate synthase 1
Short name:
PGP synthase 1
Gene namesi
Name:PGS1
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 17

Organism-specific databases

HGNCiHGNC:30029. PGS1.

Subcellular locationi

GO - Cellular componenti

  • endoplasmic reticulum Source: LIFEdb
  • mitochondrial inner membrane Source: BHF-UCL
  • mitochondrion Source: BHF-UCL
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Organism-specific databases

PharmGKBiPA142671180.

Polymorphism and mutation databases

BioMutaiPGS1.
DMDMi121942206.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 2828MitochondrionSequence analysisAdd
BLAST
Chaini29 – 556528CDP-diacylglycerol--glycerol-3-phosphate 3-phosphatidyltransferase, mitochondrialPRO_0000337105Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei49 – 491PhosphoserineBy similarity

Keywords - PTMi

Phosphoprotein

Proteomic databases

EPDiQ32NB8.
MaxQBiQ32NB8.
PaxDbiQ32NB8.
PeptideAtlasiQ32NB8.
PRIDEiQ32NB8.
TopDownProteomicsiQ32NB8-1. [Q32NB8-1]
Q32NB8-2. [Q32NB8-2]
Q32NB8-3. [Q32NB8-3]

PTM databases

iPTMnetiQ32NB8.
PhosphoSiteiQ32NB8.

Expressioni

Gene expression databases

BgeeiENSG00000087157.
CleanExiHS_PGS1.
ExpressionAtlasiQ32NB8. baseline and differential.
GenevisibleiQ32NB8. HS.

Organism-specific databases

HPAiHPA022526.

Interactioni

Protein-protein interaction databases

BioGridi114871. 1 interaction.
IntActiQ32NB8. 3 interactions.
MINTiMINT-1400418.
STRINGi9606.ENSP00000262764.

Structurei

3D structure databases

ProteinModelPortaliQ32NB8.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini215 – 24127PLD phosphodiesterase 1PROSITE-ProRule annotationAdd
BLAST
Domaini460 – 49334PLD phosphodiesterase 2PROSITE-ProRule annotationAdd
BLAST

Sequence similaritiesi

Contains 2 PLD phosphodiesterase domains.PROSITE-ProRule annotation

Keywords - Domaini

Repeat, Transit peptide

Phylogenomic databases

eggNOGiKOG3964. Eukaryota.
COG1502. LUCA.
GeneTreeiENSGT00390000002373.
HOVERGENiHBG057228.
InParanoidiQ32NB8.
KOiK00995.
OMAiEGWGTWH.
OrthoDBiEOG091G0DQT.
PhylomeDBiQ32NB8.
TreeFamiTF314768.

Family and domain databases

InterProiIPR016270. PLipase-D_PtdSer-synthase-type.
IPR001736. PLipase_D/transphosphatidylase.
[Graphical view]
PANTHERiPTHR12586. PTHR12586. 1 hit.
PROSITEiPS50035. PLD. 1 hit.
[Graphical view]

Sequences (4)i

Sequence statusi: Complete.

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

This entry describes 4 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q32NB8-1) [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
MAVAAAAAAG PVFWRRLLGL LPGRPGLAAL LGRLSDRLGR NRDRQRRRSP
60 70 80 90 100
WLLLAPLLSP AVPQVTSPPC CLCPEGVHRF QWIRNLVPEF GVSSSHVRVL
110 120 130 140 150
SSPAEFFELM KGQIRVAKRR VVMASLYLGT GPLEQELVDC LESTLEKSLQ
160 170 180 190 200
AKFPSNLKVS ILLDFTRGSR GRKNSRTMLL PLLRRFPEQV RVSLFHTPHL
210 220 230 240 250
RGLLRLLIPE RFNETIGLQH IKVYLFDNSV ILSGANLSDS YFTNRQDRYV
260 270 280 290 300
FLQDCAEIAD FFTELVDAVG DVSLQLQGDD TVQVVDGMVH PYKGDRAEYC
310 320 330 340 350
KAANKRVMDV INSARTRQQM LHAQTFHSNS LLTQEDAAAA GDRRPAPDTW
360 370 380 390 400
IYPLIQMKPF EIQIDEIVTE TLLTEAERGA KVYLTTGYFN LTQAYMDLVL
410 420 430 440 450
GTRAEYQILL ASPEVNGFFG AKGVAGAIPA AYVHIERQFF SEVCSLGQQE
460 470 480 490 500
RVQLQEYWRR GWTFHAKGLW LYLAGSSLPC LTLIGSPNFG YRSVHRDLEA
510 520 530 540 550
QIAIVTENQA LQQQLHQEQE QLYLRSGVVS SATFEQPSRQ VKLWVKMVTP

LIKNFF
Length:556
Mass (Da):62,730
Last modified:December 6, 2005 - v1
Checksum:iC6263E6F7D9B2462
GO
Isoform 2 (identifier: Q32NB8-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     112-137: Missing.
     469-480: LWLYLAGSSLPC → QFTGTWRPRLRS
     481-556: Missing.

Show »
Length:454
Mass (Da):51,306
Checksum:i383490EF68CB6A63
GO
Isoform 3 (identifier: Q32NB8-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     328-330: SNS → RTL
     331-556: Missing.

Note: No experimental confirmation available.
Show »
Length:330
Mass (Da):37,212
Checksum:iE4E48DD7BB208BEE
GO
Isoform 4 (identifier: Q32NB8-4) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     48-50: RSP → RGR
     51-556: Missing.

Note: No experimental confirmation available.
Show »
Length:50
Mass (Da):5,463
Checksum:i937C583532218D16
GO

Sequence cautioni

The sequence AAH08903 differs from that shown. Reason: Erroneous initiation. Curated
The sequence AAH15570 differs from that shown. Reason: Erroneous initiation. Curated
The sequence AAH35662 differs from that shown. Reason: Frameshift at position 420. Curated
The sequence AAH35662 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.Curated
The sequence BAB14921 differs from that shown. Reason: Erroneous translation. Wrong choice of CDS.Curated

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei48 – 503RSP → RGR in isoform 4. 1 PublicationVSP_033897
Alternative sequencei51 – 556506Missing in isoform 4. 1 PublicationVSP_033898Add
BLAST
Alternative sequencei112 – 13726Missing in isoform 2. 1 PublicationVSP_033899Add
BLAST
Alternative sequencei328 – 3303SNS → RTL in isoform 3. 1 PublicationVSP_033900
Alternative sequencei331 – 556226Missing in isoform 3. 1 PublicationVSP_033901Add
BLAST
Alternative sequencei469 – 48012LWLYL…SSLPC → QFTGTWRPRLRS in isoform 2. 1 PublicationVSP_033902Add
BLAST
Alternative sequencei481 – 55676Missing in isoform 2. 1 PublicationVSP_033903Add
BLAST

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK024529 mRNA. Translation: BAB14921.1. Sequence problems.
AK316147 mRNA. Translation: BAH14518.1.
CH471099 Genomic DNA. Translation: EAW89524.1.
BC008903 mRNA. Translation: AAH08903.2. Different initiation.
BC015570 mRNA. Translation: AAH15570.2. Different initiation.
BC025951 mRNA. Translation: AAH25951.2.
BC035662 mRNA. Translation: AAH35662.1. Sequence problems.
BC108732 mRNA. Translation: AAI08733.1.
AL359590 mRNA. Translation: CAB94876.1.
CR749720 mRNA. Translation: CAH18487.1.
CCDSiCCDS42391.1. [Q32NB8-1]
PIRiT50620.
RefSeqiNP_077733.3. NM_024419.4. [Q32NB8-1]
UniGeneiHs.654671.

Genome annotation databases

EnsembliENST00000262764; ENSP00000262764; ENSG00000087157. [Q32NB8-1]
ENST00000589425; ENSP00000465278; ENSG00000087157. [Q32NB8-4]
ENST00000589426; ENSP00000468431; ENSG00000087157. [Q32NB8-2]
GeneIDi9489.
KEGGihsa:9489.
UCSCiuc002jvm.4. human. [Q32NB8-1]

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK024529 mRNA. Translation: BAB14921.1. Sequence problems.
AK316147 mRNA. Translation: BAH14518.1.
CH471099 Genomic DNA. Translation: EAW89524.1.
BC008903 mRNA. Translation: AAH08903.2. Different initiation.
BC015570 mRNA. Translation: AAH15570.2. Different initiation.
BC025951 mRNA. Translation: AAH25951.2.
BC035662 mRNA. Translation: AAH35662.1. Sequence problems.
BC108732 mRNA. Translation: AAI08733.1.
AL359590 mRNA. Translation: CAB94876.1.
CR749720 mRNA. Translation: CAH18487.1.
CCDSiCCDS42391.1. [Q32NB8-1]
PIRiT50620.
RefSeqiNP_077733.3. NM_024419.4. [Q32NB8-1]
UniGeneiHs.654671.

3D structure databases

ProteinModelPortaliQ32NB8.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi114871. 1 interaction.
IntActiQ32NB8. 3 interactions.
MINTiMINT-1400418.
STRINGi9606.ENSP00000262764.

PTM databases

iPTMnetiQ32NB8.
PhosphoSiteiQ32NB8.

Polymorphism and mutation databases

BioMutaiPGS1.
DMDMi121942206.

Proteomic databases

EPDiQ32NB8.
MaxQBiQ32NB8.
PaxDbiQ32NB8.
PeptideAtlasiQ32NB8.
PRIDEiQ32NB8.
TopDownProteomicsiQ32NB8-1. [Q32NB8-1]
Q32NB8-2. [Q32NB8-2]
Q32NB8-3. [Q32NB8-3]

Protocols and materials databases

DNASUi9489.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000262764; ENSP00000262764; ENSG00000087157. [Q32NB8-1]
ENST00000589425; ENSP00000465278; ENSG00000087157. [Q32NB8-4]
ENST00000589426; ENSP00000468431; ENSG00000087157. [Q32NB8-2]
GeneIDi9489.
KEGGihsa:9489.
UCSCiuc002jvm.4. human. [Q32NB8-1]

Organism-specific databases

CTDi9489.
GeneCardsiPGS1.
HGNCiHGNC:30029. PGS1.
HPAiHPA022526.
MIMi614942. gene.
neXtProtiNX_Q32NB8.
PharmGKBiPA142671180.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG3964. Eukaryota.
COG1502. LUCA.
GeneTreeiENSGT00390000002373.
HOVERGENiHBG057228.
InParanoidiQ32NB8.
KOiK00995.
OMAiEGWGTWH.
OrthoDBiEOG091G0DQT.
PhylomeDBiQ32NB8.
TreeFamiTF314768.

Enzyme and pathway databases

UniPathwayiUPA00084; UER00503.
BioCyciMetaCyc:HS01560-MONOMER.
ReactomeiR-HSA-1483148. Synthesis of PG.

Miscellaneous databases

ChiTaRSiPGS1. human.
GenomeRNAii9489.
PROiQ32NB8.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000087157.
CleanExiHS_PGS1.
ExpressionAtlasiQ32NB8. baseline and differential.
GenevisibleiQ32NB8. HS.

Family and domain databases

InterProiIPR016270. PLipase-D_PtdSer-synthase-type.
IPR001736. PLipase_D/transphosphatidylase.
[Graphical view]
PANTHERiPTHR12586. PTHR12586. 1 hit.
PROSITEiPS50035. PLD. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiPGPS1_HUMAN
AccessioniPrimary (citable) accession number: Q32NB8
Secondary accession number(s): B7ZA32
, Q8IYK9, Q8TA85, Q96A75, Q9H7G9, Q9NPW7
Entry historyi
Integrated into UniProtKB/Swiss-Prot: May 20, 2008
Last sequence update: December 6, 2005
Last modified: September 7, 2016
This is version 95 of the entry and version 1 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

Complete proteome, Reference proteome

Documents

  1. Human chromosome 17
    Human chromosome 17: 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. 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.