Rabbit Polyclonal Antibody to BCKDHB (N-term)
货号：
P34718
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
抗体类型：
Primary antibody
Swissprot：
P21953
规格：
目录价
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Description |
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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 |
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|---|---|
| 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 |
| Antibody Type | Primary antibody |
| 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 antibody in PBS with 0.05% sodium azide. |
Application |
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|---|---|
| WB | 1/1000 |
| FCM | 1/25 |
Product Image
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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 Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution. 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).
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