PDP1 pyruvate dehyrogenase phosphatase catalytic subunit 1 [Homo sapiens (human)]

Gene Symbol	PDP1
Entrez Gene ID	54704
Full Name	pyruvate dehyrogenase phosphatase catalytic subunit 1
Synonyms	FLJ32517, FLJ56179, MGC119646, PDH, PDP, PDPC, PPM2C
Gene Type	protein-coding
Organism	Homo sapiens (human)
Genome	Chromosome:8 Map Location:8q22.1
Summary	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 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. Mutation in this gene causes pyruvate dehydrogenase phosphatase deficiency. Multiple alternatively spliced transcript variants encoding different isoforms have been identified.[provided by RefSeq].
Disorder	MIM: 605993 Pyruvate dehydrogenase phosphatase deficiency, 608782 (3)

mRNA	NM_018444, 239985429
Protein	NP_060914, 45439339
Product	[Pyruvate dehydrogenase [acetyl-transferring]]-phosphatase 1, mitochondrial isoform 3 precursor
RefSeq Status	REVIEWED
mRNA	NM_001161778, 239985419
Protein	NP_001155250, 239985420
Product	[Pyruvate dehydrogenase [acetyl-transferring]]-phosphatase 1, mitochondrial isoform 1
RefSeq Status	REVIEWED
mRNA	NM_001161781, 239985427
Protein	NP_001155253, 239985428
Product	[Pyruvate dehydrogenase [acetyl-transferring]]-phosphatase 1, mitochondrial isoform 3 precursor
RefSeq Status	REVIEWED
mRNA	NM_001161779, 239985421
Protein	NP_001155251, 239985422
Product	[Pyruvate dehydrogenase [acetyl-transferring]]-phosphatase 1, mitochondrial isoform 2
RefSeq Status	REVIEWED
mRNA	NM_001161780, 239985425
Protein	NP_001155252, 239985426
Product	[Pyruvate dehydrogenase [acetyl-transferring]]-phosphatase 1, mitochondrial isoform 2
RefSeq Status	REVIEWED

WikiPathways

WP78

TCA Cycle

Reactome

REACT_2071	Pyruvate metabolism
REACT_12528	Regulation of pyruvate dehydrogenase (PDH) complex
REACT_1046	Pyruvate metabolism and Citric Acid (TCA) cycle

Homo sapiens (human)	PDP1	NP_001155250.1
Pan troglodytes (chimpanzee)	PDP1	XP_528191.2
Canis lupus familiaris (dog)	PDP1	XP_535129.2
Bos taurus (cattle)	PDP1	NP_776374.1
Mus musculus (house mouse)	Pdp1	NP_001028625.1
Rattus norvegicus (Norway rat)	Pdp1	NP_062245.1
Gallus gallus (chicken)	PPM2C	XP_425932.2
Danio rerio (zebrafish)	zgc:174908	NP_001104628.1
Drosophila melanogaster (fruit fly)	Pdp	NP_572404.1
Caenorhabditis elegans	phosphatase	NP_491357.1

Process

ID	Name	Evidence
GO:0006090	pyruvate metabolic process	TAS
GO:0006470	protein dephosphorylation	IEA
GO:0010510	regulation of acetyl-CoA biosynthetic process from pyruvate	EXP

Component

ID	Name	Evidence
GO:0005739	mitochondrion	IEA
GO:0005759	mitochondrial matrix	TAS
GO:0008287	protein serine/threonine phosphatase complex	IEA

Function

ID	Name	Evidence
GO:0000287	magnesium ion binding	IEA
GO:0004724	magnesium-dependent protein serine/threonine phosphatase activity	IEA
GO:0004741	[pyruvate dehydrogenase (lipoamide)] phosphatase activity	IEA
GO:0016787	hydrolase activity	IEA
GO:0032403	protein complex binding	IEA

RefSeq Accession	Definition	Sequence	Price	Select
NM_018444	Homo sapiens pyruvate dehyrogenase phosphatase catalytic subunit 1 (PDP1), nuclear gene encoding mitochondrial protein, transcript variant 5, mRNA.	Full Length	$1501.85
NM_018444		ORF Sequence	$468.06
NM_001161778	Homo sapiens pyruvate dehyrogenase phosphatase catalytic subunit 1 (PDP1), nuclear gene encoding mitochondrial protein, transcript variant 1, mRNA.	Full Length	$1486.80
NM_001161778		ORF Sequence	$519.39
NM_001161781	Homo sapiens pyruvate dehyrogenase phosphatase catalytic subunit 1 (PDP1), nuclear gene encoding mitochondrial protein, transcript variant 4, mRNA.	Full Length	$1481.90
NM_001161781		ORF Sequence	$468.06
NM_001161779	Homo sapiens pyruvate dehyrogenase phosphatase catalytic subunit 1 (PDP1), nuclear gene encoding mitochondrial protein, transcript variant 2, mRNA.	Full Length	$1530.55
NM_001161779		ORF Sequence	$489.81
NM_001161780	Homo sapiens pyruvate dehyrogenase phosphatase catalytic subunit 1 (PDP1), nuclear gene encoding mitochondrial protein, transcript variant 3, mRNA.	Full Length	$1486.45
NM_001161780		ORF Sequence	$489.81