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PDHX pyruvate dehydrogenase complex, component X [Homo sapiens (human)]


RefSeq Accession Definition Services Price Order
NM_001135024 Homo sapiens pyruvate dehydrogenase complex, component X (PDHX), transcript variant 2, mRNA. ORF Sequence $423.69
Peptide Services
Antibody Services
Protein Services
NM_001166158 Homo sapiens pyruvate dehydrogenase complex, component X (PDHX), nuclear gene encoding mitochondrial protein, transcript variant 3, mRNA. ORF Sequence $239.25
Peptide Services
Antibody Services
Protein Services
NM_003477 Homo sapiens pyruvate dehydrogenase complex, component X (PDHX), nuclear gene encoding mitochondrial protein, transcript variant 1, mRNA. ORF Sequence $436.74
Peptide Services
Antibody Services
Protein Services


Gene Symbol PDHX
Entrez Gene ID 8050
Full Name pyruvate dehydrogenase complex, component X
Synonyms DLDBP, E3BP, OPDX, PDX1, proX
Gene Type protein-coding
Organism Homo sapiens (human)
Genome

11

11p13

Summary The pyruvate dehydrogenase (PDH) complex is located in the mitochondrial matrix and catalyzes the conversion of pyruvate to acetyl coenzyme A. The PDH complex thereby links glycolysis to Krebs cycle. The PDH complex contains three catalytic subunits, E1, E2, and E3, two regulatory subunits, E1 kinase and E1 phosphatase, and a non-catalytic subunit, E3 binding protein (E3BP). This gene encodes the E3 binding protein subunit; also known as component X of the pyruvate dehydrogenase complex. This protein tethers E3 dimers to the E2 core of the PDH complex. Defects in this gene are a cause of pyruvate dehydrogenase deficiency which results in neurological dysfunction and lactic acidosis in infancy and early childhood. This protein is also a minor antigen for antimitochondrial antibodies. These autoantibodies are present in nearly 95% of patients with the autoimmune liver disease primary biliary cirrhosis (PBC). In PBC, activated T lymphocytes attack and destroy epithelial cells in the bile duct where this protein is abnormally distributed and overexpressed. PBC eventually leads to cirrhosis and liver failure. Alternative splicing results in multiple transcript variants encoding distinct isoforms.[provided by RefSeq, Oct 2009].
Disorder

MIM: 608769

Lacticacidemia due to PDX1 deficiency, 245349 (3)

mRNA Protein Product Sequence Price Select
NM_001135024, 203098815 NP_001128496, 203098816 pyruvate dehydrogenase protein X component, mitochondrial isoform 2 Full Length $771.98
ORF Sequence $423.69
NM_001166158, 260898738 NP_001159630, 260898739 pyruvate dehydrogenase protein X component, mitochondrial isoform 3 precursor Full Length $669.90
ORF Sequence $239.25
NM_003477, 203098752 NP_003468, 203098753 pyruvate dehydrogenase protein X component, mitochondrial isoform 1 precursor Full Length $867.39
ORF Sequence $436.74
KEGG
hsa_M00307Pyruvate oxidation, pyruvate => acetyl-CoA
WikiPathways
WP534Glycolysis and Gluconeogenesis
WP78TCA Cycle
Reactome
REACT_111083The citric acid (TCA) cycle and respiratory electron transport
REACT_12528Regulation of pyruvate dehydrogenase (PDH) complex
REACT_1046Pyruvate metabolism and Citric Acid (TCA) cycle
REACT_111217Metabolism
REACT_2071Pyruvate metabolism
Homo sapiens (human)PDHXNP_003468.2
Pan troglodytes (chimpanzee)PDHXXP_001149489.1
Macaca mulatta (Rhesus monkey)PDHXXP_001109997.2
Canis lupus familiaris (dog)PDHXXP_533153.2
Bos taurus (cattle)PDHXNP_001069219.1
Mus musculus (house mouse)PdhxNP_780303.1
Gallus gallus (chicken)PDHXNP_001026358.1
Danio rerio (zebrafish)pdhxNP_956854.1
GeneCards PDHX
PDB 2F5Z, 2DNC, 1ZY8, 2F60
UniProt E9PB14, O00330
MIM 608769
Ensembl ENSG00000110435
HGNC 21350
HPRD 02002

GeneRIFs: Gene References Into Functions What's a GeneRIF?

General protein information

Preferred Names
pyruvate dehydrogenase protein X component, mitochondrial
Names
pyruvate dehydrogenase protein X component, mitochondrial
pyruvate dehydrogenase complex, E3-binding protein subunit
lipoyl-containing pyruvate dehydrogenase complex component X
pyruvate dehydrogenase complex, lipoyl-containing component X
dihydrolipoamide dehydrogenase-binding protein of pyruvate dehydrogenase complex

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