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Anti-PDHA1 Antibody (1V504) 是一种 Rabbit 抗体,靶向 PDHA1。Anti-PDHA1 Antibody (1V504) 可用于 ICC/IF。
Anti-PDHA1 Antibody (1V504) 是一种 Rabbit 抗体,靶向 PDHA1。Anti-PDHA1 Antibody (1V504) 可用于 ICC/IF。
产品描述 | Anti-PDHA1 Antibody (1V504) is a Rabbit antibody targeting PDHA1. Anti-PDHA1 Antibody (1V504) can be used in ICC/IF. |
Ig Type | Monoclonal Rabbit IgG |
克隆号 | 1V504 |
交叉反应 | Human |
验证活性 | Immunofluorescence staining of PDHA1 in HeLa cells. Cells were fixed with 4% PFA, permeabilzed with 0.1% Triton X-100 in PBS,blocked with 10% serum, and incubated with rabbit anti-Human PDHA1 monoclonal antibody (dilution ratio 1:60) at 4°C overnight. Then cells were stained with the Alexa Fluor®488-conjugated Goat Anti-rabbit IgG secondary antibody (green) and counterstained with DAPI (blue).Positive staining was localized to Cytoplasm. |
应用 | ICC/IF |
推荐剂量 | ICC-IF: 1:20-1:100 |
抗体种类 | Monoclonal |
宿主来源 | Rabbit |
构建方式 | This antibody was obtained from a rabbit immunized with purified, recombinant Human PDHA1 (rh PDHA1; P08559-1Phe30-Ser390). |
纯化方式 | Protein A |
性状 | Liquid |
缓冲液 | 0.2 μm filtered solution in PBS |
研究背景 | PDHA1, also known as C54G1, is an alpha subunit of pyruvate dehydrogenase. Pyruvate dehydrogenase, together with dihydrolipoamide acetyltransferase and lipoamide dehydrogenase, composes the pyruvate dehydrogenase (PDH) complex. The 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. PDHA1 plays a key role in the function of the PDH complex. Defects in PDHA1 can cause pyruvate dehydrogenase E1-alpha deficiency. Defects in PDHA1 also are the cause of X-linked Leigh syndrome (X-LS). X-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. |
偶联 | Unconjugated |
免疫原 | Recombinant Human PDHA1 Protein |
抗原种属 | Human |
储存方式 | Store at 2°C-8°C for 1 month. Store at -20°C or -80°C for 12 months. Avoid repeated freeze-thaw cycles. Preservative-Free. |
运输方式 | Shipping with blue ice. |
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