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线粒体内膜转位酶8A/耳聋/肌张力障碍肽抗体
本产品不向个人销售,仅用作科学研究,不用于任何人体实验及非科研性质的动物实验。

产品名称
Anti-TIMM8A
Anti-translocase of inner mitochondrial membrane 8A Antibody
产品介绍
靶标:

TIMM8A


产品别名:

DDP; DDP1; DFN1; MTS; TIM8; TIMM8A; translocase of inner mitochondrial membrane 8A; translocase of inner mitochondrial membrane 8A; mitochondrial import inner membrane translocase subunit Tim8 A; X-linked deafness dystonia protein; deafness dystonia protein 1; deafness/dystonia peptide; translocase of inner mitochondrial membrane 8 homolog A; 线粒体内膜转位酶8A/耳聋/肌张力障碍肽;


背景信息:
Mitochondrial import inner membrane translocase subunit Tim8 A, also known as Deafness-dystonia peptide or protein is an enzyme that in humans is encoded by the TIMM8A gene. This translocase is involved in the import and insertion of hydrophobic membrane proteins from the cytoplasm into the mitochondrial inner membrane. The gene is mutated in Mohr-Tranebjaerg syndrome/Deafness Dystonia Syndrome (MTS/DDS) and it is postulated that MTS/DDS is a mitochondrial disease caused by a defective mitochondrial protein import system. Defects in this gene also cause Jensen syndrome; an X-linked disease with opticoacoustic nerve atrophy and muscle weakness. This protein, along with TIMM13, forms a 70 kDa heterohexamer. Alternative splicing results in multiple transcript variants encoding distinct isoforms.

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。