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- bs-11490P
- 2025年10月16日
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500ug
| 产品编号 | bs-11490P |
| 英文名称 | TOR1A Antibody Blocking Peptide |
| 中文名称 | 扭转蛋白A封闭多肽 |
| 英文别名 | DQ2; Dystonia 1; Dystonia 1 protein; Dyt1; Tor1a; Torsin A; Torsin family 1 member A; TOR1A_HUMAN. |
| 纯化方法 | HPLC |
| 研究领域 | Neuroscience > Neurology process > Growth and Development > Axonal Guidance Proteins Neuroscience > Neurology process > Neurodegenerative disease > Alzheimer's disease Neuroscience > Neurology process > Neurodegenerative disease > Parkinson's disease Neuroscience > Neurology process > Neurogenesis |
| 亚基 | May form homohexamers. Interacts with TOR1AIP1 and TOR1AIP2. Interacts with KLHL14, preferentially when ATP-free. |
| 亚细胞定位 | Endoplasmic reticulum lumen. Nucleus membrane. Note=Mainly located in the lumen of the endoplasmic reticulum. The association with nuclear envelope is mediated by the interaction with TOR1AIP2. The Glu-303 del variant is lumenally-oriented in discrete large spheroid intracellular structures rather than in the endoplasmic reticulum. |
| 组织特异性 | Widely expressed. Highest levels in kidney and liver. Not detected in spleen. In the brain, high levels found in the dopaminergic neurons of the substantia nigra pars compacta, as well as in the neocortex, hippocampus and cerebellum. Also high expression in the spinal cord. |
| 相似性 | Belongs to the clpA/clpB family. Torsin subfamily. |
| 功能 | May serve as a molecular chaperone assisting in the proper folding of secreted and/or membrane proteins. In the nucleus, displaces the nuclear membrane proteins SUN2, SYNE2 and nesprin-3/C14orf49, leaving nuclear pores and SUN1 unchanged. |
| 保存条件 | Shipped at 4℃. Stored at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 背景资料 | A mutation of the DYT1 gene, which codes for TorsinA, has been identified as the cause of one form of autosomal dominantly inherited dystonia. Early-onset torsion dystonia is a movement disorder, characterized by twisting muscle contractures, that begins in childhood. Symptoms are believed to result from altered neuronal communication in the basal ganglia. TorsinA comprises 332 amino acids. TorsinA is widely expressed throughout the mouse central nervous system and is detected in the majority of neurons in nearly all regions. The proteins display cytoplasmic distribution, although in some types of neurons localization is perinuclear. TorsinA often performs chaperone-like functions that assist in the assembly, operation, or dis-assembly of protein complexes. The gene which encodes TorsinA has high homology to three additional mammalian genes and a nematode gene and distal similarity to the family of heat-shock proteins and the Clp protease family. The gene which encodes TorsinA maps to human chromosome 9q34. |
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TOR1A Antibody Blocking Peptide(bs-11490P)-500ug
￥880
