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BCKDHB抗体
BCKDHB抗体图片
交货期:1周


产品名称
Anti-BCKDHB
Anti-branched chain keto acid dehydrogenase E1 subunit beta Antibody
产品介绍
靶标:

BCKDHB


产品别名:

BCKDE1B; BCKDH E1-beta; E1B; BCKDHB; branched chain keto acid dehydrogenase E1 subunit beta; branched chain keto acid dehydrogenase E1 subunit beta; 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial; E1b-beta subunit of the branched-chain complex; branched chain alpha-ketoacid dehydrogenase E1-beta subunit; branched chain keto acid dehydrogenase E1 beta; branched chain keto acid dehydrogenase E1, beta polypeptide; branched-chain alpha-keto acid dehydrogenase E1 component beta chain; testis secretory sperm-binding protein Li 240mP;


背景信息:
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.

宿主:Rbt
类型:Pab
同种型:IgG  
应用:WB
纯化方式:亲和纯化
偶联物:Unconjugated
性状:液体
存储溶液:参阅说明书
浓度:Batch dependent (Please refer to the vial label for the specific concentration.)
稀释比例: Optimal dilutions/concentrations should be determined by the end user           
储存:经常使用则4°C保存。-20°C保存不超过两年。避免反复冻融。
注意事项:仅供实验室使用。不适用于人类或动物的任何临床,治疗或诊断用途。不适合动物或人类食用。

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