Rabbit Anti-PHE1B/PDH-E1 beta antibody

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Pyruvate Dehydrogenase E1 beta subunit; PDH-E1β; DKFZp564K0164; mitochondrial; ODPB_HUMAN; pdhB; PDHBD; PDHE1 B; PDHE1-B; PHE1B; Pyruvate dehydrogenase (lipoamide) beta; Pyruvate dehydrogenase E1 beta polypeptide; Pyruvate dehydrogenase E1 compo

Cat:

SL19694R

Species Reactivity：

(predicted: Human,Mouse,Rat,)

Immunogen：

KLH conjugated synthetic peptide derived from human PHE1B/PDH-E1 beta:101-200/359

Format：

Liquid

Storage instructions：

Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

ELISA=1:5000-10000IHC-P=1:100-500IHC-F=1:100-500ICC=1:100-500IF=1:100-500（Paraffin sections need to do antigen repair）not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

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Unit:

Price: $228.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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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. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Mar 2012]

Function:
The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2). It contains multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3).

Subunit:
Heterotetramer of two PDHA1 and two PDHB subunits. The heterotetramer interacts with DLAT, and is part of the multimeric pyruvate dehydrogenase complex that contains multiple copies of pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (DLAT, E2) and lipoamide dehydrogenase (DLD, E3). These subunits are bound to an inner core composed of about 48 DLAT and 12 PDHX molecules.

Subcellular Location:
Mitochondrion matrix.

DISEASE:
Defects in PDHB are a cause of pyruvate dehydrogenase E1 component deficiency (PDHE1 deficiency) [MIM:312170]. PDHE1 deficiency is the most common enzyme defect in patients with primary lactic acidosis. It is associated with variable clinical phenotypes ranging from neonatal death to prolonged survival complicated by developmental delay, seizures, ataxia, apnea, and in some cases to an X-linked form of Leigh syndrome (LS) (Leigh encephalomyelopathy).

SWISS:
P11177

Gene ID:
5162

Database links:

Entrez Gene: 5162 Human

Entrez Gene: 68263 Mouse

Entrez Gene: 289950 Rat

Omim: 179060 Human

SwissProt: P11966 Cow

SwissProt: P11177 Human

SwissProt: Q9D051 Mouse

SwissProt: P49432 Rat

Unigene: 161357 Human

Unigene: 301527 Mouse

Unigene: 102424 Rat

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