Name: U-Blot® Pyruvate Dehydrogenase E1 α Rabbit mAb
SKU: WR4169
Price: 268 USD
Availability: InStock

U-Blot® Pyruvate Dehydrogenase E1 α Rabbit mAb

Various whole cell lysates were separated by 4-20% SDS-PAGE, and the primary antibody was used at 4°C, over night with a 1:2500 dilution . The Dylight 800-conjugated Goat anti-Rabbit antibody(Cat:WS0446) was used to detect the antibody.

WR4169 Various whole cell lysates were separated by 4-20% SDS-PAGE, and the primary antibody was used at 4°C, over night with a 1:2500 dilution . The Dylight 800-conjugated Goat anti-Rabbit antibody(Cat:WS0446) was used to detect the antibody.

Immunohistochemical analysis of paraffin-embedded Rat kidney tissue. 1, primary Antibody was diluted at 1:200(4～C,overnight). 2, EDTA pH 9.0 was used for antibody retrieval(>98～C,20min). 3,Secondary antibody was diluted at 1:200(room tempeRature, 30min).

WR4169 Immunohistochemical analysis of paraffin-embedded Rat kidney tissue. 1, primary Antibody was diluted at 1:200(4～C,overnight). 2, EDTA pH 9.0 was used for antibody retrieval(>98～C,20min). 3,Secondary antibody was diluted at 1:200(room tempeRature, 30min).

Immunohistochemical analysis of paraffin-embedded Mouse kidney tissue. 1, primary Antibody was diluted at 1:200(4～C,overnight). 2, EDTA pH 9.0 was used for antibody retrieval(>98～C,20min). 3,Secondary antibody was diluted at 1:200(room tempeRature, 30min).

WR4169 Immunohistochemical analysis of paraffin-embedded Mouse kidney tissue. 1, primary Antibody was diluted at 1:200(4～C,overnight). 2, EDTA pH 9.0 was used for antibody retrieval(>98～C,20min). 3,Secondary antibody was diluted at 1:200(room tempeRature, 30min).

Immunohistochemical analysis of paraffin-embedded Human kidney tissue. 1, primary Antibody was diluted at 1:200(4～C,overnight). 2, EDTA pH 9.0 was used for antibody retrieval(>98～C,20min). 3,Secondary antibody was diluted at 1:200(room tempeRature, 30min).

WR4169 Immunohistochemical analysis of paraffin-embedded Human kidney tissue. 1, primary Antibody was diluted at 1:200(4～C,overnight). 2, EDTA pH 9.0 was used for antibody retrieval(>98～C,20min). 3,Secondary antibody was diluted at 1:200(room tempeRature, 30min).

Western blot analysis of lysates from HEK293 cells, using Pyruvate Dehydrogenase E1 α Rabbit mAb. The HRP-conjugated Goat anti-Rabbit IgG antibody (WS0002) was used to detect the antibody.

WR4169 Western blot analysis of lysates from HEK293 cells, using Pyruvate Dehydrogenase E1 α Rabbit mAb. The HRP-conjugated Goat anti-Rabbit IgG antibody (WS0002) was used to detect the antibody.

Price range: $268.00 through $328.00

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50μl

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SKU: WR4169-50

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Product details

Background: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],

Specifications

Size	50μL/100μL
Target	PDHA1
Reactivity	Human, Mouse, Rat
Applications	WB, IHC, IF, IP, ELISA
MW(Calculated)	43kD
MW(Observed)	43kD
Host Species	Rabbit
Isotype	IgG,Kappa
Conjugate/Modification	Unmodified
Recommended Dilution Ratio	IHC 1:200-1:1000; WB 1:1000-1:5000; IF 1:200-1:1000; ELISA 1:5000-1:20000; IP 1:50-1:200, / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004)
Formulation	PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
Purification	Recombinant Antibody expressed in animal component-free (ACF) media, purified via Protein A affinity chromatography.
Storage	-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration
Clonality	Monoclonal
Clone Number	RM0322R
Immunogen	The specific immunogen used to produce this antibody is proprietary information.
Specificity	Endogenous
Gene Name	PDHA1 PHE1A
Protein Name	PDHA1 ODPA
Other Name	PDHA1; / PHE1A; / Pyruvate dehydrogenase E1 component subunit alpha; / somatic form, mitochondrial; / PDHE1-A type I
Species	Human
Gene ID	5160
Uniprot	P08559,
Species.1	Mouse
Gene ID.1	18597
Uniprot.1	P35486,
Species.2	Rat
Gene ID.2
Uniprot.2	P26284
Cellular Localization	Mitochondrion matrix
Tissue Expression	Ubiquitous.
Signaling_pathway	Organismal Systems >> Endocrine system >> Glucagon signaling pathway;Human Diseases >> Cancer: overview >> Central carbon metabolism in cancer;Environmental Information Processing >> Signal transduction >> HIF-1 signaling pathway
Research Areas
Function	Catalytic activity:Pyruvate + [dihydrolipoyllysine-residue acetyltransferase] lipoyllysine = [dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine + CO(2).,cofactor:Thiamine pyrophosphate.,Disease:Defects in PDHA1 are a cause of pyruvate decarboxylase 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).,Disease:Defects in PDHA1 are the cause of X-linked Leigh syndrome (LS) [MIM:308930]. LS is an early-onset progressive neurodegenerative disorder with a characteristic neuropathology consisting of focal, bilateral lesions in one or more areas of the central nervous system, including the brainstem, thalamus, basal ganglia, cerebellum, and spinal cord. The lesions are areas of demyelination, gliosis, necrosis, spongiosis, or capillary proliferation. Clinical symptoms depend on which areas of the central nervous system are involved. The most common underlying cause is a defect in oxidative phosphorylation. LS may be a feature of a deficiency of any of the mitochondrial respiratory chain complexes.,enzyme regulation:E1 activity is regulated by phosphorylation (inactivation) and dephosphorylation (activation) of the alpha subunit.,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:Tetramer of 2 alpha and 2 beta subunits.,tissue specificity:Ubiquitous.,