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Rabbit anti Human Aprataxin
Rabbit anti Human Aprataxin
- 中文名称：
- Rabbit anti Human Aprataxin
- 英文名称：
- Rabbit anti Human Aprataxin
- 品牌：
- Nordic-MUbio
- 品牌介绍：
- Nordic公司成立于1964年，是荷兰著名免疫学试剂生产厂商，拥有超过1700种针对人、小鼠、猴和大鼠的免疫球蛋白及其亚型的特异性抗血清。公司主要产品包括一系列广泛的针对不同物种血清蛋白的多克隆抗血清，以及针对酶的一系列独特的多克隆抗血清。
- 货号：
- X2725P
- 规格：
- 100 µg
- 保存建议：
- -20ºC. Aliquot to avoid freeze/thaw cycles
- 背景资料：
- DNA-binding protein involved in single-strand DNA break repair, double-strand DNA break repair and base excision repair. Resolves abortive DNA ligation intermediates formed either at base excision sites, or when DNA ligases attempt to repair non-ligatable breaks induced by reactive oxygen species. Catalyzes the release of adenylate groups covalently linked to 5'-phosphate termini, resulting in the production of 5'-phosphate termini that can be efficiently rejoined. Also able to hydrolyze adenosine 5'-monophosphoramidate (AMP-NH2) and diadenosine tetraphosphate (AppppA), but with lower catalytic activity. Protein is widely expressed. Defects in APTX are the cause of ataxia-oculomotor apraxia syndrome, an autosomal recessive syndrome characterized by early-onset cerebellar ataxia, oculomotor apraxia, early areflexia and late peripheral neuropathy. Also a cause of coenzyme Q10 deficiency. Coenzyme Q10 deficiency is an autosomal recessive disorder with variable manifestations. It can be associated with three main clinical phenotypes: a predominantly myopathic form with central nervous system involvement, an infantile encephalomyopathy with renal dysfunction and an ataxic form with cerebellar atrophy. Coenzyme Q10 deficiency due to APTX mutations is typically associated with cerebellar ataxia.
- 货期：
- 现货/4周
- 来源宿主：
- Rabbit
- 应用：
- Western Blotting|ELISA
- 免责声明：
- *本产品仅供科研实验使用，不得用于临床诊断。*
- 其他：
别名 / 同义词:Forkhead-associated domain histidine triad-like protein; FHA-HIT; APTX; AXA1
克隆号/Clone:N/A
亚型/Isotype:N/A
研究领域/Field of Interest:Signal Transduction
标记/Label:N/A
- 说明书：
