中文 
别名:2-oxoisovalerate dehydrogenase subunit beta, mitochondrial, Branched-chain alpha-keto acid dehydrogenase E1 component beta chain, BCKDE1B, BCKDH E1-beta, BCKDHB应用:WB,FCM
反应种属:Human, Rat
规格：50μl/100μl
| Description |
|---|
| Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). This gene encodes the E1 beta subunit, and mutations therein have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation, and feeding problems. |
| Specification | |
|---|---|
| Aliases | 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial, Branched-chain alpha-keto acid dehydrogenase E1 component beta chain, BCKDE1B, BCKDH E1-beta, BCKDHB |
| Entrez GeneID | 594 |
| Swissprot | P21953 |
| WB Predicted band size | 43.1kDa |
| Host/Isotype | Rabbit IgG |
| Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| Species Reactivity | Human, Rat |
| Immunogen | This BCKDHB antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 41-70 amino acids from the N-terminal region of human BCKDHB. |
| Formulation | Purified polyclonal antibody supplied in PBS with 0.05% sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification. |
| Application | |
|---|---|
| WB | 1/1000 |
| FCM | 1/25 |
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All lanes : Anti-BCKDHB Antibody (N-term) at 1:1000 dilution Lane 1: Hela whole cell lysate Lane 2: Jurkat whole cell lysate Lane 3: Daudi whole cell lysate Lysates/proteins at 20 µg per lane. Secondary Predicted band size : 43 kDa Blocking/Dilution buffer: 5% NFDM/TBST. |
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Immunofluorescent analysis of 4% paraformaldehyde-fixed, 0. 1% Triton X-100 permeabilized Hela cells labeling BCKDHB with P34718 at 1/25 dilution, followed by Dylight® 488-conjugated goat anti-Rabbit IgG secondary antibody at 1/200 dilution (green). Immunofluorescence image showing Cytoplasm staining on Hela cell line. The nuclear counter stain is DAPI (blue). |
本公司的所有产品仅用于科学研究或者工业应用等非医疗目的，不可用于人类或动物的临床诊断或治疗，非药用，非食用。
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本公司的所有产品仅用于科学研究或者工业应用等非医疗目的，不可用于人类或动物的临床诊断或治疗，非药用，非食用。
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