PPCS - phosphopantothenoylcysteine synthetase Gene

Also Known as CMD2C

生物種: Homo sapiens

遺伝子タイプ: protein coding
遺伝子ID: 79717

About PPCS

Cytogenetic location: 1p34.2 Genomic coordinates (GRCh38): 1:42,456,341-42,473,417 (from NCBI)

This gene has 8 transcripts (splice variants), 193 orthologues, 4 paralogues and is associated with 2 phenotypes. Ubiquitous expression in kidney (RPKM 13.6), thyroid (RPKM 13.4) and 25 other tissues.

Summary

Biosynthesis of coenzyme A (CoA) from pantothenic acid (vitamin B5) is an essential universal pathway in prokaryotes and eukaryotes. PPCS (EC 6.3.2.5), one of the last Enzymes in this pathway, converts phosphopantothenate to phosphopantothenoylcysteine (Daugherty et al., 2002 [PubMed 11923312]).[supplied by OMIM, Mar 2008]

PPCS Products (8)

mRNA Protein Name
NM_001077447.3 NP_001070915.1 phosphopantothenate--cysteine ligase isoform b
NM_001287506.1 NP_001274435.1 phosphopantothenate--cysteine ligase isoform b
NM_001287507.1 NP_001274436.1 phosphopantothenate--cysteine ligase isoform c
NM_001287508.2 NP_001274437.1 phosphopantothenate--cysteine ligase isoform b
NM_001287509.2 NP_001274438.1 phosphopantothenate--cysteine ligase isoform b
NM_001287510.2 NP_001274439.1 phosphopantothenate--cysteine ligase isoform b
NM_001287511.2 NP_001274440.1 phosphopantothenate--cysteine ligase isoform d
NM_024664.4 NP_078940.2 phosphopantothenate--cysteine ligase isoform a

Gene Ontology

  • Molecular Function
  • Biological Process
Molecular Function GO Annotation Evidence 参考文献 由来
enables identical protein binding IPI
IPI: Inferred from physical interaction
32296183 GOA
enables phosphopantothenate--cysteine ligase activity IDA
IDA: Inferred from direct assay
11923312 GOA
enables phosphopantothenate--cysteine ligase activity IMP
IMP: Inferred from mutant phenotype
29754768 GOA
enables protein homodimerization activity IMP
IMP: Inferred from mutant phenotype
29754768 GOA
Biological Process GO Annotation Evidence 参考文献 由来
involved in acetyl-CoA biosynthetic process IMP
IMP: Inferred from mutant phenotype
29754768 GOA
involved in coenzyme A biosynthetic process IDA
IDA: Inferred from direct assay
11923312 GOA
involved in coenzyme A biosynthetic process IMP
IMP: Inferred from mutant phenotype
29754768 GOA
involved in heart process IMP
IMP: Inferred from mutant phenotype
29754768 GOA
EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

PPCS Protein Structure

DFP

DFP: DNA / pantothenate metabolism flavoprotein (36 - 83)

DFP

DFP: DNA / pantothenate metabolism flavoprotein (158 - 264)

  • 0
  • 100
  • 200
  • 311 a.a.
Protein Preferred Names Protein Names

phosphopantothenate--cysteine ligase

  • PPC synthetase

PPCS Protein-protein interaction Information

Type
タンパク質名 Protein ID Interactor Interactor Species Interactor ID Detection Method 参考文献
Intra
PPCS Q9HAB8 PDZRN3 Homo sapiens Q9UPQ7
Anti Tag CoIP
33961781
Intra
PPCS Q9HAB8 LACRT Homo sapiens Q9GZZ8
Anti Tag CoIP
33961781
Intra
PPCS Q9HAB8 SCGB2A1 Homo sapiens O75556
Anti Tag CoIP
33961781
Intra
PPCS Q9HAB8 BPIFB1 Homo sapiens Q8TDL5
Anti Tag CoIP
33961781
Intra
PPCS Q9HAB8 HP Homo sapiens P00738
Anti Tag CoIP
33961781
Intra
PPCS Q9HAB8 LTF Homo sapiens P02788
Anti Tag CoIP
33961781
Intra
PPCS Q9HAB8 DMBT1 Homo sapiens Q9UGM3
Anti Tag CoIP
33961781
Intra
PPCS Q9HAB8 PPCS Homo sapiens Q9HAB8
Y2H Prey Pooling
32296183
Intra
PPCS Q9HAB8 a0a6l8p747_bacan Bacillus anthracis A0A6L8P747
Y2H Pooling
20711500
Intra
PPCS Q9HAB8 PPCS Homo sapiens Q9HAB8
Validated Y2H
32296183
Intra
PPCS Q9HAB8 PPCS Homo sapiens Q9HAB8
Y2H Array
32296183
Intra
PPCS Q9HAB8 IGHA2 Homo sapiens P01877
Anti Tag CoIP
33961781
Cross: Cross-species interaction Intra: Intraspecies interaction

PPCS 抗体

製品番号 製品名 アプリケーション 反応性
HY-P83432 PPCS Antibody (YA3177) WB, IP Human
HY-P83432A PPCS Antibody (YA3177)(PBS only) WB, IP Human

関連疾患

Diseases Alias
Cardiomyopathy, Dilated, 2c

  • CMD2C

  • Dilated Cardiomyopathy 2c

  • Cardiomyopathy, Dilated 2c
Familial Isolated Dilated Cardiomyopathy

  • Familial Or Idiopathic Dilated Cardiomyopathy
Bladder Tuberculosis

  • Tuberculous Cystitis

  • Tuberculosis Of Bladder
Periosteal Chondrosarcoma

  • Juxtacortical Chondrosarcoma
Dilated Cardiomyopathy

  • Familial Dilated Cardiomyopathy

  • Primary Dilated Cardiomyopathy

  • Idiopathic Dilated Cardiomyopathy

  • Congestive Cardiomyopathy

  • Idiopathic Dilation Cardiomyopathy

  • Primary Familial Dilated Cardiomyopathy

  • Cardiomyopathy, Dilated

  • DCM

  • Cardiomyopathy, Familial Dilated

  • Dilated Cardiomyopathy, Familial

  • Hypokinetic Dilated Cardiomyopathy, Familial

  • Familial Idiopathic Cardiomyopathy

  • Fdc

  • Cardiomyopathy, Familial Idiopathic

  • Idiopathic Cardiomegaly

  • Dilated Congestive Cardiomyopathy

  • Chronic Dilated Cardiomyopathy

  • Ccm - [Congestive Cardiomyopathy]

  • Cocm - [Congestive Cardiomyopathy]

  • Dcm - [Dilated Cardiomyopathy]

  • Dilated-Hypokinetic Cardiomyopathy

  • Congestive Idiopathic Cardiomyopathy

  • Primary Idiopathic Dilated Cardiomyopathy
Urethral Calculus

  • Calculus In Urethra

  • Urethral Stone

  • Urethrolithiasis

  • Urethra Calculi Impaction

  • Urethra Calculus Impaction

  • Urethra Stone

  • Calculous Urethritis

  • Urethral Calculi Impaction

  • Urethral Calculus Impaction
Neurodegeneration With Brain Iron Accumulation 1

  • Pantothenate Kinase-Associated Neurodegeneration

  • Pkan

  • NBIA1

  • Hallervorden-Spatz Disease

  • Hallervorden-Spatz Syndrome

  • Pigmentary Pallidal Degeneration

  • Neuroaxonal Dystrophy, Late Infantile

  • Neurodegeneration With Brain Iron Accumulation Type 1

  • Classic Pantothenate Kinase-Associated Neurodegeneration

  • Pkan Neuroaxonal Dystrophy, Juvenile-Onset

  • Brain Iron Accumulation Type I Syndrome

  • Nbia

  • Neurodegeneration With Brain Iron Accumulation

  • Nbia1, Classic Form

  • Neurodegeneration With Brain Iron Accumulation Type 1, Classic Form

  • Pkan, Classic Form

  • Atypical Pantothenate Kinase-Associated Neurodegeneration

  • Nbia1, Atypical Form

  • Neurodegeneration With Brain Iron Accumulation Type 1, Atypical Form

  • Pkan, Atypical Form

  • Hss

  • Pkan Neuroaxonal Dystrophy Juvenile-Onset

  • Neurodegeneration, With Brain Iron Accumulation, Type 1
Diseases Alias
Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Related Agents

  • Clinical Phase
  • Pre-clinical Phase

Clinical Phase

Bioactive Molecules (1)
製品番号 製品名 製品効果
純度 Phase
HY-109105 Tepilamide fumarate 99.77% Phase 2

Pre-clinical Phase

Bioactive Molecules (7)
製品番号 製品名
製品効果
純度
Rare Diseases
HY-W071006 PPC-NHS ester 98.0% Unkown
HY-144691 PP-C8 Inhibitor 99.99% Unkown
HY-126530 PPC-NB 99.90% Unkown
HY-117843 Ppc-1 / Unkown
HY-176791 CDK12-Cyclin K Ligand-Linker Conjugates 1 Inhibitor / Unkown
HY-RS10935 PPCS Human Pre-designed siRNA Set A Pre-designed Set / Unkown
HY-176790 CDK12-Cyclin K ligand-1 Inhibitor / Unkown

Orthologs Information

生物種 Symbol 由来 ID
Mus musculus PPCS MGD MGI:1915237
Bos taurus PPCS VGNC VGNC:33189
Felis catus PPCS VGNC VGNC:64307
Canis familiaris PPCS VGNC VGNC:44841
Rattus norvegicus PPCS RGD RGD:1311399
Macaca mulatta PPCS VGNC VGNC:76112
Others PPCS NCBI