全部产品分类
品牌 |
Leading Biology | 货号 |
APR13775G |
产品分类 |
Monoclonal Antibodies | 研究领域 |
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产品概述 |
We constantly strive to ensure we provide our customers with the best antibodies. As a result of this work we offer this antibody in purified format.
We are in the process of updating our datasheets. If you have any questions regarding this update, please feel free to contact our technical support team.
This product is a high quality TOR1A antibody.
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分子量 |
37809 Da
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细胞定位 |
Antigen Cellular Localization:
Endoplasmic reticulum lumen. Nucleus membrane; Peripheral membrane protein. Cell projection, growth cone. Cytoplasmic vesicle membrane Cytoplasmic vesicle, secretory vesicle. Cytoplasmic vesicle, secretory vesicle, synaptic vesicle. Cytoplasm, cytoskeleton. Note=Upon oxidative stress, redistributes to protusions from the cell surface (By similarity). Peripherally associated with the inner face of the ER membrane, probably mediated by the interaction with TOR1AIP1. The association with nucleus membrane is mediated by the interaction with TOR1AIP2
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宿主 |
Rabbit
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种属反应性 |
Human
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靶点 |
A synthetic peptide corresponding to residues near the C terminus of human TorsinA was used as an immunogen.
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克隆号 |
EP2569Y
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通用名 |
DQ2, DYT1, TA, TORA
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基因ID |
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UniProt ID |
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功能 |
Protein with chaperone functions important for the control of protein folding, processing, stability and localization as well as for the reduction of misfolded protein aggregates. Involved in the regulation of synaptic vesicle recycling, controls STON2 protein stability in collaboration with the COP9 signalosome complex (CSN). In the nucleus, may link the cytoskeleton with the nuclear envelope, this mechanism seems to be crucial for the control of nuclear polarity, cell movement and, specifically in neurons, nuclear envelope integrity. Participates in the cellular trafficking and may regulate the subcellular location of multipass membrane proteins such as the dopamine transporter SLC6A3, leading to the modulation of dopamine neurotransmission. In the endoplasmic reticulum, plays a role in the quality control of protein folding by increasing clearance of misfolded proteins such as SGCE variants or holding them in an intermediate state for proper refolding. May have a redundant function with TOR1B in non- neural tissues.
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总结 |
TorsinA is an AAA(+) protein that has been demonstrated as an endoplasmic reticulum (ER) chaperone protein involved in a sensitive reporter system for quantitation of processing through the secretory pathway. It is predominantly located in the lumen of the ER and nuclear envelope. TorsinA is responsible for early onset torsion dystonia (DYT1 dystonia), a dominantly inherited movement disorder and disease of basal ganglia function. DYT1 dystonia is commonly caused by the deletion of a glutamic acid (DeltaE) in the carboxyl terminal region of TorsinA, where the protein then aggregates in perinuclear inclusions instead of the ER (1-3). TorsinA is degraded primarily through the macroautophagy?lysosome pathway, whereas the TorsinA DeltaE mutant protein is degraded by both the proteasome and macroautophagy?lysosome pathways (4).
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形式 |
50 mM Tris-Glycine (pH 7.4), 0.15 M NaCl, 40% Glycerol, 0.01% sodium azide and 0.05% BSA.
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储存条件 |
Store at +4°C short term. For long-term storage, aliquot and store at -20°C or below. Stable for 12 months at -20°C. Avoid repeated freeze-thaw cycles.
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应用 |
WB
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稀释方法 |
WB~~1:500~1000
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别名 |
Torsin-1A, Dystonia 1 protein, Torsin ATPase-1A, 364-, Torsin family 1 member A, TOR1A, DQ2, DYT1, TA, TORA
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图像 |
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说明书 |
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数量 |
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