FITC标记的囊泡相关膜蛋白相关的蛋白B
产品名称： FITC标记的囊泡相关膜蛋白相关的蛋白B
英文名称： Anti-VAPB/FITC
产品编号： HZ-
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： IF=1:50-200
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Rabbit Anti-VAPB/FITC Conjugated antibody
FITC标记的囊泡相关膜蛋白相关的蛋白B
| 英文名称 | Anti-VAPB/FITC |
| 中文名称 | FITC标记的囊泡相关膜蛋白相关的蛋白B |
| 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 B; VAMP B VAMP C; VAMP B/VAMP C; VAMP C; VAMP vesicle associated membrane protein associated protein B and C; Vamp33b; VAMPB; VAMPB/VAMPC; VAMPC; VAP 33b; VAP B; VAP B/VAP C; VAP C; VAP33b; VAPB/VAPC; VAPC antibody Vesicle associated membrane protein associated protein B and C; Vesicle associated membrane protein associated protein B/C; VAPB_HUMAN. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 肿瘤 细胞生物 免疫学 信号转导 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Chicken, Pig, Cow, Horse, Rabbit, Sheep, |
| 产品应用 | IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 27kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human VAPB |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 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 adult 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 adult 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 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, adult, 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, onset in late adulthood (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: Entrez Gene: 9217 Human Entrez Gene: 56491 Mouse Entrez Gene: 60431 Rat Omim: 605704 Human SwissProt: O95292 Human SwissProt: Q9QY76 Mouse SwissProt: Q9Z269 Rat Unigene: 182625 Human Unigene: 260456 Mouse Unigene: 52500 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
VAB含有1个MSP结构域，可能在囊泡运输中起作用。VAPB中的缺陷是近端常染色体显性脊髓性肌萎缩症[MIM：182980 ]的原因；也称为迟发性脊髓性肌萎缩芬克尔型。脊髓性肌萎缩症是神经退行性疾病，其特征是下运动神经元变性，导致进行性麻痹性肌萎缩。这种形式是一种迟发性发病形式（20岁以后）。患者表现为良性的病程，大多数患者在临床发作后10至40年内仍保持行走。