囊泡相关膜蛋白相关的蛋白B
规格：1mg/1ml
英文名： VAPB
别名： ALS 8; ALS8; D2Abb2e; UNQ484/PRO983; Vamp 33b; VAMP associated 33 kDa protein; VAMP associated protein B and C; VAMP associated protein B; VAMP associated protein B/C; VAMP associated protein C; VAMP
分子量： 27kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human VAPB
交叉反应：Human, Mouse, Rat, Chicken, Pig, Cow, Horse, Rabbit, Sheep,
细胞定位：
囊泡相关膜蛋白相关的蛋白B产品介绍：background: VAPB contains 1 MSP domain and it may play a role in vesicle trafficking. Defects in VAPB are a cause of proximal ***** autosomal dominant spinal muscular atrophy [MIM:182980]; also called late onset spinal muscular atrophy Finkel type. Spinal muscular atrophies are neurodegenerative disorders characterized by degeneration of lower motor neurons, leading to progressive paralysis muscular atrophy. This form is a late ***** onset form of the disease (after age 20 years). The patients show a benign course, most of them remaining ambulatory 10 to 40 years after clinical onset. Function: Participates in the endoplasmic reticulum unfolded protein response (UPR) by inducing ERN1/IRE1 activity. Involved in cellular calcium homeostasis regulation. Subunit: Homodimer, and heterodimer with VAPA. Interacts with VAMP1 and VAMP2. Interacts with HCV NS5A and NS5B. Interacts (via MSP domain) with ZFYVE27. Interacts with RMDN3. Subcellular Location: Endoplasmic reticulum membrane; Single-pass type IV membrane protein (By similarity). Note=Present in mitochondria-associated membranes that are endoplasmic reticulum membrane regions closely apposed to the outer mitochondrial membrane. Tissue Specificity: Ubiquitous. Isoform 1 predominates. DISEASE: Amyotrophic lateral sclerosis 8 (ALS8) [MIM:608627]: A neurodegenerative disorder affecting upper motor neurons囊泡相关膜蛋白相关的蛋白B in the brain and lower motor neurons in the brain stem and spinal cord, resulting in fatal paralysis. Sensory abnormalities are absent. The pathologic hallmarks of the disease include pallor of the corticospinal tract due to loss of motor neurons, presence of ubiquitin-positive inclusions within surviving motor neurons, and deposition of pathologic aggregates. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of the cases. Note=The disease is caused by mutations affecting the gene represented in this entry. Spinal muscular atrophy, proximal, *****, autosomal dominant (SMAPAD) [MIM:182980]: A form of spinal muscular atrophy, a neuromuscular disorder characterized by degeneration of the anterior horn cells of the spinal cord, leading to symmetrical muscle weakness and atrophy. SMAPAD is characterized by proximal muscle weakness that begins in the lower limbs and then progresses to upper limbs, 囊泡相关膜蛋白相关的蛋白Bonset in late *****hood (after third decade) and a benign course. Most of the patients remain ambulatory 10 to 40 years after clinical onset. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the VAMP-associated protein (VAP) (TC 9.B.17) family. Contains 1 MSP domain. Database links: UniProtKB/Swiss-Prot: O95292.3 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
囊泡相关膜蛋白相关的蛋白B产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 **学 信号转导
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid