PDHA1

The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial multienzyme complex that catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and provides the primary link between glycolysis and the tricarboxylic acid (TCA) cycle. The PDH complex is composed of multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). The E1 enzyme is a heterotetramer of two alpha and two beta subunits. This gene encodes the E1 alpha 1 subunit containing the E1 active site, and plays a key role in the function of the PDH complex. Mutations in this gene are associated with pyruvate dehydrogenase E1-alpha deficiency and X-linked Leigh syndrome. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Mar 2010]
Full Name
Pyruvate Dehydrogenase E1 Alpha 1 Subunit
Function
The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO2, and thereby links the glycolytic pathway to the tricarboxylic cycle.
Biological Process
Acetyl-CoA biosynthetic process from pyruvateManual Assertion Based On ExperimentIDA:UniProtKB
Glucose metabolic processIEA:UniProtKB-KW
Mitochondrial acetyl-CoA biosynthetic process from pyruvateIEA:Ensembl
Tricarboxylic acid cycleIEA:UniProtKB-KW
Cellular Location
Mitochondrion matrix
Involvement in disease
Pyruvate dehydrogenase E1-alpha deficiency (PDHAD):
An enzymatic defect causing primary lactic acidosis in children. It is associated with a broad clinical spectrum ranging from fatal lactic acidosis in the newborn to chronic neurologic dysfunction with structural abnormalities in the central nervous system without systemic acidosis.
PTM
Phosphorylation at Ser-232, Ser-293 and Ser-300 by PDK family kinases inactivates the enzyme; for this phosphorylation at a single site is sufficient. Dephosphorylation at all three sites, i.e. at Ser-232, Ser-293 and Ser-300, is required for reactivation.
Acetylation alters the phosphorylation pattern. Deacetylated by SIRT3 (By similarity).
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Anti-PDHA1 antibodies

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Mouse Anti-PDHA1 Recombinant Antibody (CF333) (CBMAB-FT168LY)

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Target: PDHA1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CF333
Application*: ELISA, WB, IHC, IF, FC, IP
Datasheet SDS

Mouse Anti-PDHA1 Recombinant Antibody (CB05A) (CBMAB-CA005LY)

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Target: PDHA1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Dog, Rat, Mouse, Pig
Clone: CB05A
Application*: ELISA, WB, ICC
Datasheet SDS

Mouse Anti-PDHA1 Recombinant Antibody (9H9AF5) (CBMAB-P1282-YC)

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Target: PDHA1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Cattle, Mouse, Rat
Clone: 9H9AF5
Application*: E, F, IC, IF, WB
Datasheet SDS

Mouse Anti-PDHA1 Recombinant Antibody (8D10E6) (CBMAB-P1281-YC)

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Target: PDHA1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Cattle, C. elegans, Fruit fly, Mouse, Rat
Clone: 8D10E6
Application*: IC, IF, WB
Datasheet SDS

Mouse Anti-PDHA1 Recombinant Antibody (2C10) (CBMAB-P1280-YC)

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Target: PDHA1
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: 2C10
Application*: F, IF, WB
Datasheet SDS

Mouse Anti-PDHA1 Recombinant Antibody (2B10) (CBMAB-P1278-YC)

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Target: PDHA1
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: 2B10
Application*: WB, IF, F
Datasheet SDS

Mouse Anti-PDHA1 Recombinant Antibody (CBT1012) (V2LY-0625-LY3110)

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Target: PDHA1
Host: Mouse
Antibody Isotype: IgG
Specificity: Human, Mouse, Rat, Cattle, Dog, Pig
Clone: CBT1012
Application*: WB, IF, IC
Datasheet SDS

Mouse Anti-PDHA1 Recombinant Antibody (CBT3471) (V2LY-0625-LY1989)

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Target: PDHA1
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human, Mouse, Rat
Clone: CBT3471
Application*: WB, IH, IC, F
Datasheet SDS

Mouse Anti-PDHA1 Recombinant Antibody (CBT4517) (V2LY-0625-LY1988)

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Target: PDHA1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Mouse, Rat
Clone: CBT4517
Application*: WB, IP, IH, IC, F
Datasheet SDS

Mouse Anti-PDHA1 Recombinant Antibody (CBT3345) (V2LY-0625-LY1920)

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Target: PDHA1
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: CBT3345
Application*: WB, F
Datasheet SDS
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(P): Predicted
* Abbreviations
  • AActivation
  • AGAgonist
  • APApoptosis
  • BBlocking
  • BABioassay
  • BIBioimaging
  • CImmunohistochemistry-Frozen Sections
  • CIChromatin Immunoprecipitation
  • CTCytotoxicity
  • CSCostimulation
  • DDepletion
  • DBDot Blot
  • EELISA
  • ECELISA(Cap)
  • EDELISA(Det)
  • ESELISpot
  • EMElectron Microscopy
  • FFlow Cytometry
  • FNFunction Assay
  • GSGel Supershift
  • IInhibition
  • IAEnzyme Immunoassay
  • ICImmunocytochemistry
  • IDImmunodiffusion
  • IEImmunoelectrophoresis
  • IFImmunofluorescence
  • IGImmunochromatography
  • IHImmunohistochemistry
  • IMImmunomicroscopy
  • IOImmunoassay
  • IPImmunoprecipitation
  • ISIntracellular Staining for Flow Cytometry
  • LALuminex Assay
  • LFLateral Flow Immunoassay
  • MMicroarray
  • MCMass Cytometry/CyTOF
  • MDMeDIP
  • MSElectrophoretic Mobility Shift Assay
  • NNeutralization
  • PImmunohistologyp-Paraffin Sections
  • PAPeptide Array
  • PEPeptide ELISA
  • PLProximity Ligation Assay
  • RRadioimmunoassay
  • SStimulation
  • SESandwich ELISA
  • SHIn situ hybridization
  • TCTissue Culture
  • WBWestern Blot
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