PDHX antibody

Este producto es parte de PDHX - Pyruvate dehydrogenase protein X
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935106861
info@markelab.com
name
PDHX antibody
category
Primary Antibodies
provider
FineTest
reference
FNab06271
tested applications
ELISA, IHC, IP, WB

Description

Required for anchoring dihydrolipoamide dehydrogenase(E3) to the dihydrolipoamide transacetylase(E2) core of the pyruvate dehydrogenase complexes of eukaryotes. This specific binding is essential for a functional PDH complex.

Documents del producto

Instrucciones
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Data sheet
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Product specifications

Category
Primary Antibodies
Immunogen Target
pyruvate dehydrogenase complex, component X (PDHX)
Host
Rabbit
Reactivity
Human, Mouse, Rat
Recommended Dilution
WB: 1:500-1:2000; IP: 1:200-1:1000; IHC: 1:20-1:200
Clonality
polyclonal
Conjugation
Unconjugated
Isotype
IgG
Observed MW
54kd
Purity
≥95% as determined by SDS-PAGE
Purification
Immunogen affinity purified
Size 1
100µg
Form
liquid
Tested Applications
ELISA, IHC, IP, WB
Storage
PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20℃ for 12 months(Avoid repeated freeze / thaw cycles.)
UniProt ID
O00330
Gene ID
8050
Alias
DLDBP, E3BP, OPDX, PDX1, proX, pyruvate dehydrogenase complex component X, PDHXD
Background
Antibody anti-PDHX
Status
RUO
Note
Mol. Weight 54 kDa

Descripción

PDHX is a component of the pyruvate dehydrogenase complex (PDC) located in the mitochondrial matrix, where it plays a critical role in linking glycolysis to the tricarboxylic acid (TCA) cycle by catalyzing the conversion of pyruvate to acetyl-CoA. PDHX functions as the E3-binding protein, anchoring dihydrolipoamide dehydrogenase (E3) to the PDC core and facilitating the transfer of reducing equivalents between PDC subunits. It is essential for the stability and activity of the PDC, ensuring efficient energy production and metabolic homeostasis. Mutations in PDHX are associated with pyruvate dehydrogenase deficiency, a condition characterized by lactic acidosis, developmental delay, and neurological dysfunction due to impaired mitochondrial energy metabolism. PDHX is expressed ubiquitously in energy-demanding tissues such as the brain, muscle, and liver, where it supports ATP production and metabolic adaptation. Knockout studies demonstrate reduced PDC activity, metabolic imbalances, and severe defects in energy-dependent processes, underscoring its importance in cellular respiration and energy metabolism.

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PDHX antibody

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Pyruvate Dehydrogenase Complex Component X (PDHX) Antibody

PDHX Antibody is a Rabbit Polyclonal antibody against PDHX. 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.

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