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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

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

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

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

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

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

Human Recombinant PDHA1 protein, His Tag (V2LY-0526-LY6067)

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Target: PDHA1

Expressed Host: E. coli

Sequence: Amino Acid: 410-end

Tag: His Tag

Mouse Anti-PDHA1 Recombinant Antibody (
12C9) (V2LY-0725-LY351)

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Target: PDHA1

Host: Mouse

Antibody Isotype: IgG1

Specificity: Human, Dog, Rat, Mouse, Pig

Clone: 12C9

Application*: E, WB, IC

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

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

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

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

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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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