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| Reactivity: | Mouse, Rat |
| Applications: | WB, ELISA |
| Host Species: | Rabbit |
| Isotype: | IgG |
| Clonality: | Polyclonal antibody |
| Gene Name: | branched chain keto acid dehydrogenase E1 subunit beta |
| Gene Symbol: | BCKDHB |
| Synonyms: | E1B; BCKDE1B; BCKDH E1-beta; BCKDHB |
| Gene ID: | 594 |
| UniProt ID: | P21953 |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 183-392 of human BCKDHB (NP_898871.1). |
| Dilution: | WB 1:1000-1:5000 |
| Purification Method: | Affinity purification |
| Concentration: | 2.47 mg/ml |
| Buffer: | PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Storage: | Store at -20°C. Avoid freeze / thaw cycles. |
| Documents: | Manual-BCKDHB antibody |
Background
This gene encodes the E1 beta subunit of branched-chain keto acid dehydrogenase, which is a multienzyme complex associated with the inner membrane of mitochondria. This enzyme complex functions in the catabolism of branched-chain amino acids. Mutations in this gene 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. Alternative splicing at this locus results in multiple transcript variants.
Images
 | Western blot analysis of various lysates, using BCKDHB Rabbit pAb (A10533) at 1:2000 dilution. Secondary antibody: HRP-conjugated Goat anti-Rabbit IgG (H+L) (AS014) at 1:10000 dilution. Lysates/proteins: 25μg per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 60s. |
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