Anti-BCKDHB Polyclonal Antibody - SAlexa Fluor 568
Cat: K112661P - SAlexa Fluor 568
Summary:
| 【Product name】：Anti-BCKDHB Polyclonal Antibody - SAlexa Fluor 568 | 【Source】：Rabbit |
| 【Isotype】：IgG | 【Purification】：Affinity purification |
| 【Gene ID】：594 | 【Swiss Prot】：P21953 |
| 【Observed】：36kDa | |
| 【Tested applications】：IHC,WB | |
| 【Recommended dilution】：WB 1:2000-5000. IHC 1:25-100. | |
| 【Immunogen】：Recombinant protein of human BCKDHB | |
| 【Public Immunogen Range】：160-393aa | |
| 【Storage】：Store at -20℃. Avoid freeze / thaw cycles. | |
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.
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