BCKDHB抗体

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BCKDHB抗体


交货期:	1周
产品别名:	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。