PDHA1

Item Information
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Description

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.

Product Overview
Entrez GenelD
5160
Aliases
PDHA; PDHAD; PHE1A; PDHCE1A
Clone#
2A2H7
Host / Isotype
Mouse / Mouse IgG2b
Species Reactivity
Human
Immunogen
Purified recombinant fragment of human PDHA1 (AA: 241-390) expressed in E. Coli.
Formulation
Purified antibody in PBS with 0.05% sodium azide
Storage
4℃; -20℃ for long term storage
Product Applications
WB (Western Blot)
1/500 - 1/2000
FCM (Flow Cytometry)
1/200 - 1/400
ELISA
1/10000
References
1.Eur J Paediatr Neurol. 2021 Mar;31:27-30.
2.Pathol Res Pract. 2019 Mar;215(3):478-482.
Product Image
Elisa
Figure 1:Black line: Control Antigen (100 ng);Purple line: Antigen (10ng); Blue line: Antigen (50 ng); Red line:Antigen (100 ng)
Figure 1:Black line: Control Antigen (100 ng);Purple line: Antigen (10ng); Blue line: Antigen (50 ng); Red line:Antigen (100 ng)
Western Blot
Figure 2:Western blot analysis using PDHA1 mAb against human PDHA1 (AA: 241-390) recombinant protein. (Expected MW is 37.5 kDa)
Figure 2:Western blot analysis using PDHA1 mAb against human PDHA1 (AA: 241-390) recombinant protein. (Expected MW is 37.5 kDa)
Western Blot
Figure 3:Western blot analysis using PDHA1 mAb against HEK293-6e (1) and PDHA1 (AA: 241-390)-hIgGFc transfected HEK293-6e (2) cell lysate.
Figure 3:Western blot analysis using PDHA1 mAb against HEK293-6e (1) and PDHA1 (AA: 241-390)-hIgGFc transfected HEK293-6e (2) cell lysate.
Western Blot
Figure 4:Western blot analysis using PDHA1 mouse mAb against HepG2 (1), HL-60 (2),SK-OV-3 (3),PC-3 (4) and PANC-1 (5) cell lysate.
Figure 4:Western blot analysis using PDHA1 mouse mAb against HepG2 (1), HL-60 (2),SK-OV-3 (3),PC-3 (4) and PANC-1 (5) cell lysate.
Immunofluorescence analysis
Figure 5:Flow cytometric analysis of Hela cells using PDHA1 mouse mAb (green) and negative control (red).
Figure 5:Flow cytometric analysis of Hela cells using PDHA1 mouse mAb (green) and negative control (red).
For Research Use Only. Not for use in diagnostic procedures.