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PDHB

The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial multienzyme complex that catalyzes the overall conversion of pyruvate to acetyl-CoA and carbon dioxide, 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 beta subunit. Mutations in this gene are associated with pyruvate dehydrogenase E1-beta deficiency.

Full Name

pyruvate dehydrogenase (lipoamide) beta

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 pyruvate1 PublicationIC:MGI
Tricarboxylic acid cycleManual Assertion Based On ExperimentTAS:ProtInc

Cellular Location

Mitochondrion matrix

Involvement in disease

Pyruvate dehydrogenase E1-beta deficiency (PDHBD):
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.

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Anti-PDHB antibodies

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Mouse Anti-PDHB Recombinant Antibody (CBYC-P263) (CBMAB-P1289-YC)

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Target: PDHB

Host: Mouse

Antibody Isotype: IgG1

Specificity: Human

Clone: CBYC-P263

Application*: E, IH, WB

Rabbit Anti-PDHB Recombinant Antibody (CBYC-P262) (CBMAB-P1288-YC)

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Target: PDHB

Host: Rabbit

Antibody Isotype: IgG

Specificity: Human

Clone: CBYC-P262

Application*: IC, IF

Mouse Anti-PDHB Recombinant Antibody (2B2) (CBMAB-P1286-YC)

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Target: PDHB

Host: Mouse

Antibody Isotype: IgG1, κ

Specificity: Human

Clone: 2B2

Application*: E, P, WB

Mouse Anti-PDHB Recombinant Antibody (1D1) (CBMAB-P1285-YC)

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Target: PDHB

Host: Mouse

Antibody Isotype: IgG2b, κ

Specificity: Human

Clone: 1D1

Application*: E

Mouse Anti-PDHB Recombinant Antibody (17A5E2H8) (CBMAB-P1284-YC)

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Target: PDHB

Host: Mouse

Antibody Isotype: IgG1

Specificity: Human, Mouse, Rat, Cattle

Clone: 17A5E2H8

Application*: WB, P

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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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