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Protein

Phosphatidylserine decarboxylase proenzyme, mitochondrial

Gene

PISD

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 4 out of 5-Experimental evidence at transcript leveli

Functioni

Catalyzes the formation of phosphatidylethanolamine (PtdEtn) from phosphatidylserine (PtdSer). Plays a central role in phospholipid metabolism and in the interorganelle trafficking of phosphatidylserine.UniRule annotation

Catalytic activityi

Phosphatidyl-L-serine = phosphatidylethanolamine + CO2.UniRule annotation

Cofactori

pyruvateUniRule annotationNote: Binds 1 pyruvoyl group covalently per subunit.UniRule annotation

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Active sitei191Charge relay system; for autoendoproteolytic cleavage activityUniRule annotation1
Active sitei267Charge relay system; for autoendoproteolytic cleavage activityUniRule annotation1
Active sitei378Charge relay system; for autoendoproteolytic cleavage activityUniRule annotation1
Active sitei378Schiff-base intermediate with substrate; via pyruvic acid; for decarboxylase activityUniRule annotation1

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Decarboxylase, Lyase

Keywords - Biological processi

Lipid biosynthesis, Lipid metabolism, Phospholipid biosynthesis, Phospholipid metabolism

Keywords - Ligandi

Pyruvate

Enzyme and pathway databases

BioCyciMetaCyc:HS01985-MONOMER.
ZFISH:HS01985-MONOMER.
ReactomeiR-HSA-1483213. Synthesis of PE.

Names & Taxonomyi

Protein namesi
Recommended name:
Phosphatidylserine decarboxylase proenzyme, mitochondrialUniRule annotation (EC:4.1.1.65UniRule annotation)
Cleaved into the following 2 chains:
Phosphatidylserine decarboxylase beta chainUniRule annotation
Phosphatidylserine decarboxylase alpha chainUniRule annotation
Gene namesi
Name:PISDUniRule annotation
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 22

Organism-specific databases

HGNCiHGNC:8999. PISD.

Subcellular locationi

Chain Phosphatidylserine decarboxylase beta chain :
  • Mitochondrion inner membrane UniRule annotation; Single-pass membrane protein UniRule annotation; Intermembrane side UniRule annotation
Chain Phosphatidylserine decarboxylase alpha chain :
  • Mitochondrion inner membrane UniRule annotation; Peripheral membrane protein UniRule annotation; Intermembrane side UniRule annotation

  • Note: Anchored to the mitochondrial inner membrane through its interaction with the integral membrane beta chain.UniRule annotation

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini53 – 63Mitochondrial matrixUniRule annotationAdd BLAST11
Transmembranei64 – 82HelicalUniRule annotationAdd BLAST19
Topological domaini83 – 409Mitochondrial intermembraneUniRule annotationAdd BLAST327

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Organism-specific databases

DisGeNETi23761.
OpenTargetsiENSG00000241878.
PharmGKBiPA33333.

Chemistry databases

DrugBankiDB00144. Phosphatidylserine.

Polymorphism and mutation databases

BioMutaiPISD.
DMDMi311033492.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 52MitochondrionSequence analysisAdd BLAST52
ChainiPRO_000043557153 – 409Phosphatidylserine decarboxylase proenzymeAdd BLAST357
ChainiPRO_000002983553 – 377Phosphatidylserine decarboxylase beta chainUniRule annotationAdd BLAST325
ChainiPRO_0000029836378 – 409Phosphatidylserine decarboxylase alpha chainUniRule annotationAdd BLAST32

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei378Pyruvic acid (Ser); by autocatalysisUniRule annotation1

Post-translational modificationi

Is synthesized initially as an inactive proenzyme. Formation of the active enzyme involves a self-maturation process in which the active site pyruvoyl group is generated from an internal serine residue via an autocatalytic post-translational modification. Two non-identical subunits are generated from the proenzyme in this reaction, and the pyruvate is formed at the N-terminus of the alpha chain, which is derived from the carboxyl end of the proenzyme. The autoendoproteolytic cleavage occurs by a canonical serine protease mechanism, in which the side chain hydroxyl group of the serine supplies its oxygen atom to form the C-terminus of the beta chain, while the remainder of the serine residue undergoes an oxidative deamination to produce ammonia and the pyruvoyl prosthetic group on the alpha chain. During this reaction, the Ser that is part of the protease active site of the proenzyme becomes the pyruvoyl prosthetic group, which constitutes an essential element of the active site of the mature decarboxylase.UniRule annotation

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sitei377 – 378Cleavage (non-hydrolytic); by autocatalysisUniRule annotation2

Keywords - PTMi

Zymogen

Proteomic databases

EPDiQ9UG56.
MaxQBiQ9UG56.
PaxDbiQ9UG56.
PeptideAtlasiQ9UG56.
PRIDEiQ9UG56.

PTM databases

iPTMnetiQ9UG56.
PhosphoSitePlusiQ9UG56.

Expressioni

Gene expression databases

BgeeiENSG00000241878.
CleanExiHS_PISD.
ExpressionAtlasiQ9UG56. baseline and differential.
GenevisibleiQ9UG56. HS.

Organism-specific databases

HPAiHPA031090.
HPA031091.

Interactioni

Subunit structurei

Heterodimer of a large membrane-associated beta subunit and a small pyruvoyl-containing alpha subunit.UniRule annotation

Protein-protein interaction databases

BioGridi117262. 4 interactors.
IntActiQ9UG56. 5 interactors.
STRINGi9606.ENSP00000266095.

Structurei

3D structure databases

ProteinModelPortaliQ9UG56.
SMRiQ9UG56.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the phosphatidylserine decarboxylase family. PSD-B subfamily. Eukaryotic type I sub-subfamily.UniRule annotation

Keywords - Domaini

Transit peptide, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG2420. Eukaryota.
COG0688. LUCA.
GeneTreeiENSGT00390000013484.
HOVERGENiHBG039630.
InParanoidiQ9UG56.
KOiK01613.
OMAiGLFCLNE.
OrthoDBiEOG091G09PP.
PhylomeDBiQ9UG56.
TreeFamiTF313148.

Family and domain databases

HAMAPiMF_03208. PS_decarb_PSD_B_type1_euk. 1 hit.
InterProiIPR003817. PS_Dcarbxylase.
IPR033177. PSD.
IPR033661. PSD_type1_euk.
[Graphical view]
PANTHERiPTHR10067. PTHR10067. 1 hit.
PfamiPF02666. PS_Dcarbxylase. 1 hit.
[Graphical view]
TIGRFAMsiTIGR00163. PS_decarb. 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: Q9UG56-3) [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
MATSVGHRCL GLLHGVAPWR SSLHPCEITA LSQSLQPLRK LPFRAFRTDA
60 70 80 90 100
RKIHTAPART MFLLRPLPIL LVTGGGYAGY RQYEKYRERE LEKLGLEIPP
110 120 130 140 150
KLAGHWEVAL YKSVPTRLLS RAWGRLNQVE LPHWLRRPVY SLYIWTFGVN
160 170 180 190 200
MKEAAVEDLH HYRNLSEFFR RKLKPQARPV CGLHSVISPS DGRILNFGQV
210 220 230 240 250
KNCEVEQVKG VTYSLESFLG PRMCTEDLPF PPAASCDSFK NQLVTREGNE
260 270 280 290 300
LYHCVIYLAP GDYHCFHSPT DWTVSHRRHF PGSLMSVNPG MARWIKELFC
310 320 330 340 350
HNERVVLTGD WKHGFFSLTA VGATNVGSIR IYFDRDLHTN SPRHSKGSYN
360 370 380 390 400
DFSFVTHTNR EGVPMRKGEH LGEFNLGSTI VLIFEAPKDF NFQLKTGQKI

RFGEALGSL
Length:409
Mass (Da):46,672
Last modified:November 2, 2010 - v4
Checksum:i6A5148265369D9DF
GO
Isoform 2 (identifier: Q9UG56-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-107: MATSVGHRCL...IPPKLAGHWE → MMCQSEARQG...SRVGWRPVSR

Note: No experimental confirmation available.
Show »
Length:375
Mass (Da):43,047
Checksum:i4ED283AD1944C3C9
GO

Sequence cautioni

The sequence CAB43678 differs from that shown. Reason: Erroneous translation.Curated

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0075401 – 107MATSV…AGHWE → MMCQSEARQGPELRAAKWLH FPQLALRRRLGQLSCMSRPA LKLRSWPLTVLYYLLPFGAL RPLSRVGWRPVSR in isoform 2. 1 PublicationAdd BLAST107

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
CR456540 mRNA. Translation: CAG30426.1.
AL050371 Transcribed RNA. Translation: CAB43678.2. Sequence problems.
AL096768 Genomic DNA. Translation: CAB56394.1.
AL096768, AL031255 Genomic DNA. Translation: CAI23032.1.
AL031255, AL096768 Genomic DNA. Translation: CAI22447.1.
CH471095 Genomic DNA. Translation: EAW59984.1.
BC001482 mRNA. Translation: AAH01482.1.
BC009315 mRNA. Translation: AAH09315.1.
AF035304 mRNA. Translation: AAB88186.1.
CCDSiCCDS13899.1. [Q9UG56-2]
RefSeqiNP_001313340.1. NM_001326411.1. [Q9UG56-3]
NP_001313344.1. NM_001326415.1. [Q9UG56-2]
NP_001313345.1. NM_001326416.1. [Q9UG56-2]
NP_001313346.1. NM_001326417.1. [Q9UG56-2]
NP_055153.1. NM_014338.3. [Q9UG56-2]
NP_821141.1. NM_178022.1. [Q9UG56-2]
UniGeneiHs.420559.
Hs.732339.

Genome annotation databases

EnsembliENST00000266095; ENSP00000266095; ENSG00000241878. [Q9UG56-2]
ENST00000382151; ENSP00000371586; ENSG00000241878. [Q9UG56-2]
ENST00000439502; ENSP00000391739; ENSG00000241878. [Q9UG56-3]
GeneIDi23761.
KEGGihsa:23761.
UCSCiuc003alk.3. human. [Q9UG56-3]

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
CR456540 mRNA. Translation: CAG30426.1.
AL050371 Transcribed RNA. Translation: CAB43678.2. Sequence problems.
AL096768 Genomic DNA. Translation: CAB56394.1.
AL096768, AL031255 Genomic DNA. Translation: CAI23032.1.
AL031255, AL096768 Genomic DNA. Translation: CAI22447.1.
CH471095 Genomic DNA. Translation: EAW59984.1.
BC001482 mRNA. Translation: AAH01482.1.
BC009315 mRNA. Translation: AAH09315.1.
AF035304 mRNA. Translation: AAB88186.1.
CCDSiCCDS13899.1. [Q9UG56-2]
RefSeqiNP_001313340.1. NM_001326411.1. [Q9UG56-3]
NP_001313344.1. NM_001326415.1. [Q9UG56-2]
NP_001313345.1. NM_001326416.1. [Q9UG56-2]
NP_001313346.1. NM_001326417.1. [Q9UG56-2]
NP_055153.1. NM_014338.3. [Q9UG56-2]
NP_821141.1. NM_178022.1. [Q9UG56-2]
UniGeneiHs.420559.
Hs.732339.

3D structure databases

ProteinModelPortaliQ9UG56.
SMRiQ9UG56.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi117262. 4 interactors.
IntActiQ9UG56. 5 interactors.
STRINGi9606.ENSP00000266095.

Chemistry databases

DrugBankiDB00144. Phosphatidylserine.

PTM databases

iPTMnetiQ9UG56.
PhosphoSitePlusiQ9UG56.

Polymorphism and mutation databases

BioMutaiPISD.
DMDMi311033492.

Proteomic databases

EPDiQ9UG56.
MaxQBiQ9UG56.
PaxDbiQ9UG56.
PeptideAtlasiQ9UG56.
PRIDEiQ9UG56.

Protocols and materials databases

DNASUi23761.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000266095; ENSP00000266095; ENSG00000241878. [Q9UG56-2]
ENST00000382151; ENSP00000371586; ENSG00000241878. [Q9UG56-2]
ENST00000439502; ENSP00000391739; ENSG00000241878. [Q9UG56-3]
GeneIDi23761.
KEGGihsa:23761.
UCSCiuc003alk.3. human. [Q9UG56-3]

Organism-specific databases

CTDi23761.
DisGeNETi23761.
GeneCardsiPISD.
HGNCiHGNC:8999. PISD.
HPAiHPA031090.
HPA031091.
MIMi612770. gene.
neXtProtiNX_Q9UG56.
OpenTargetsiENSG00000241878.
PharmGKBiPA33333.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG2420. Eukaryota.
COG0688. LUCA.
GeneTreeiENSGT00390000013484.
HOVERGENiHBG039630.
InParanoidiQ9UG56.
KOiK01613.
OMAiGLFCLNE.
OrthoDBiEOG091G09PP.
PhylomeDBiQ9UG56.
TreeFamiTF313148.

Enzyme and pathway databases

BioCyciMetaCyc:HS01985-MONOMER.
ZFISH:HS01985-MONOMER.
ReactomeiR-HSA-1483213. Synthesis of PE.

Miscellaneous databases

ChiTaRSiPISD. human.
GeneWikiiPISD_(gene).
GenomeRNAii23761.
PROiQ9UG56.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000241878.
CleanExiHS_PISD.
ExpressionAtlasiQ9UG56. baseline and differential.
GenevisibleiQ9UG56. HS.

Family and domain databases

HAMAPiMF_03208. PS_decarb_PSD_B_type1_euk. 1 hit.
InterProiIPR003817. PS_Dcarbxylase.
IPR033177. PSD.
IPR033661. PSD_type1_euk.
[Graphical view]
PANTHERiPTHR10067. PTHR10067. 1 hit.
PfamiPF02666. PS_Dcarbxylase. 1 hit.
[Graphical view]
TIGRFAMsiTIGR00163. PS_decarb. 1 hit.
ProtoNetiSearch...

Entry informationi

Entry nameiPISD_HUMAN
AccessioniPrimary (citable) accession number: Q9UG56
Secondary accession number(s): B1AKM7
, O43207, O95535, Q6IC28, Q96GQ2, Q9UGA9
Entry historyi
Integrated into UniProtKB/Swiss-Prot: May 30, 2003
Last sequence update: November 2, 2010
Last modified: November 30, 2016
This is version 136 of the entry and version 4 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 22
    Human chromosome 22: 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. 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.