This gene encodes a hexameric DNA helicase which unwinds short stretches of double-stranded DNA in the 5' to 3' direction and, along with mitochondrial single-stranded DNA binding protein and mtDNA polymerase gamma, is thought to play a key role in mtDNA replication. The protein localizes to the mitochondrial matrix and mitochondrial nucleoids. Mutations in this gene cause infantile onset spinocerebellar ataxia (IOSCA) and progressive external ophthalmoplegia (PEO) and are also associated with several mitochondrial depletion syndromes. Alternative splicing results in multiple transcript variants encoding distinct isoforms.
应用类型
WB
免疫原
The immunogen is a synthetic peptide directed towards the C-terminal region of Human PEO1
来源宿主
Rabbit
反应性
Human
保存建议
For short term use, store at 2-8C up to 1 week. For long term storage, store at -20C in small aliquots to prevent freeze-thaw cycles.
其他
Aviva Systems Biology总部位于加利福尼亚州圣迭戈，在中国北京设有办公室，专注于为研究需求提供多克隆和单克隆抗体、ELISA试剂盒、蛋白质和定制服务。Aviva Systems Biology生产了24,000种经过验证的多克隆抗体，并提供近20,000种ELISA试剂盒，定制实验室服务包括蛋白表达和纯化、抗体开发，以及ELISA的开发、验证和生产。Aviva Systems Biology为与独特物种和靶标相关的研究提供独特工具，研究领域包括转录因子、癌症、心血管、细胞生物学、DNA损伤和修复、表观遗传学、信号转导、细胞分化、干细胞生物学等等。
