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产品名称:Anti-TIMM8A抗体优货,线粒体内膜转位酶8A/肌张力障碍肽抗体
中文名称:线粒体内膜转位酶8A/肌张力障碍肽抗体
英文名称:Anti-TIMM8A
产品编号:BYK-11769R
产品别名:DDP 1; DDP; DDP1; Deafness dystonia protein 1; Deafness/dystonia peptide; DFN 1; DFN1; MGC12262; Mitochondrial import inner membrane translocase subunit Tim8 A; MTS; TIM 8A; TIM8A; TIMM 8A; Translocase of inner mitochondrial membrane 8 homolog A; X linked deafness dystonia protein; TIM8A_HUMAN.
本公司另供应"Anti-TIMM8A抗体优货,线粒体内膜转位酶8A/肌张力障碍肽抗体”的标记有:Alexa Fluor 350 Alexa Fluor 488 Alexa Fluor 555 Alexa Fluor 647 AP APC Biotin Cy3 Cy5 Cy5.5 Cy7 FITC Gold HRP PE PE-Cy3 PE-CY5 PE-CY5.5 PE-CY7 RBITC.
产品规格:100ug/200ug
产品浓度: 1mg/1ml
抗体来源:兔源、鼠源、山羊
克 隆:单克隆抗体、多克隆抗体
抗体类型:一抗
性 状: 冻干粉或液体
保存条件:Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
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产品介绍:
The majority of mitochondrial-directed proteins are encoded by the nuclear genome and are transported to the mitochondria via regulated processes involving the mitochondrial Tom and Tim proteins (1). The mitochondrial Tim protein family is comprised of a large group of evolutionarily conserved proteins that are found in most eukaryotes (1,2). Import of nuclear-encoded precursor proteins into and across the mitochondrial inner membrane is mediated by two distinct complexes, the Tim23 complex and the Tim22 complex, which differ in their substrate specificity (1). Defects in Tim proteins are implicated in several neuro-degenerative diseases, suggesting important roles for Tim proteins in development and health (3,4). Tim8A and Tim8B, which map to human chromosomes Xq22.1 and 11q23.1-q23.2, respectively, are conserved proteins of the mitochondrial intermembrane space, which are organized in hetero-oligomeric complex with Tim13 (5,6,7). Tim8A is highly expressed in fetal and adult brain (5). Tim8A is mutated in deafness dystonia syndrome, a novel type of disease that causes severe neurological defects, thought to be caused by a defective mitochondrial protein transport system (5,8).