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Pyruvate dehydrogenase phosphatase

Template:Infobox3cols/rowTemplate:Infobox3cols/rowTemplate:Infobox3cols/rowTemplate:Infobox3cols/row
Identifiers
SymbolsPDP1 ; PDH; PDP; PDPC; PPM2C
External IDsOMIM605993 MGI2685870 HomoloGene31928 GeneCards: PDP1 Gene
EC number3.1.3.43
Orthologs
SpeciesHumanMouse
Entrez54704381511
EnsemblENSG00000164951ENSMUSG00000049225
UniProtQ9P0J1Q3UV70
RefSeq (mRNA)NM_001161778NM_001033453
RefSeq (protein)NP_001155251NP_001028625
Location (UCSC)Chr 8:
94.87 – 94.94 Mb
Chr 4:
11.96 – 11.97 Mb
PubMed search[1][2]

pyruvate dehyrogenase phosphatase catalytic subunit 1 (PDPC 1), also known as protein phosphatase 2C, is an enzyme that in humans is encoded by the PDP1gene.[1][2] PDPC 1 is an enzyme which serves to reverse the effects of pyruvate dehydrogenase kinase upon pyruvate dehydrogenase.

Function

Pyruvate dehydrogenase (E1) is one of the three components (E1, E2, and E3) of the large pyruvate dehydrogenase complex. Pyruvate dehydrogenase kinases catalyze phosphorylation of serine residues of E1 to inactivate the E1 component and inhibit the complex. Pyruvate dehydrogenase phosphatases catalyze the dephosphorylation and activation of the E1 component to reverse the effects of pyruvate dehydrogenase kinases. Pyruvate dehydrogenase phosphatase is a heterodimer consisting of catalytic and regulatory subunits. Two catalytic subunits have been reported; one is predominantly expressed in skeletal muscle and another one is much more abundant in the liver. The catalytic subunit, encoded by this gene, is the former, and belongs to the protein phosphatase 2C (PP2C) superfamily. Along with the pyruvate dehydrogenase complex and pyruvate dehydrogenase kinases, this enzyme is located in the mitochondrial matrix.[1]

Regulation

Pyruvate dehydrogenase phosphatase is stimulated by insulin, PEP, and AMP, but competitively inhibited by ATP, NADH, and Acetyl-CoA.

Clinical significance

Mutation in this gene causes pyruvate dehydrogenase phosphatase deficiency.[1]

References

  1. ^ a b c "Entrez Gene: pyruvate dehyrogenase phosphatase catalytic subunit 1". 
  2. ^ Lawson JE, Niu XD, Browning KS, Trong HL, Yan J, Reed LJ (September 1993). "Molecular cloning and expression of the catalytic subunit of bovine pyruvate dehydrogenase phosphatase and sequence similarity with protein phosphatase 2C". Biochemistry 32 (35): 8987–93. PMID 8396421. doi:10.1021/bi00086a002. 

Further reading

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External links

This article incorporates text from the United States National Library of Medicine, which is in the public domain.



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