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Rabbit Anti-RAB7/BF647 Conjugated antibody (bs-6703R-BF647)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-6703R-BF647
英文名称1 Rabbit Anti-RAB7/BF647 Conjugated antibody
中文名称 BF647标记的RAS癌基因相关蛋白RAB7抗体
别    名 CMT2B; Member RAS oncogene family; PSN; RAB7A; RAB7B; Ras associated protein RAB7; Ras related protein Rab7; Ras related protein Rab7a; RAB7A_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  信号转导  细胞类型标志物  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse,  (predicted: Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
产品应用 Flow-Cyt=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 23kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human RAB7
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:
Members of the RAB family of RAS related GTP binding proteins are important regulators of vesicular transport and are located in specific intracellular compartments. RAB7 has been localized to late endosomes and shown to be important in the late endocytic pathway. In addition, it has been shown to have a fundamental role in the cellular vacuolation induced by the cytotoxin VacA of Helicobacter pylori.

Function:
Involved in late endocytic transport. Contributes to the maturation of phagosomes (acidification).

Subunit:
Interacts with RILP. Interacts with PSMA7. Interacts with RNF115. Interacts with FYCO1

Subcellular Location:
Late endosome. Lysosome. Cytoplasmic vesicle, phagosome. Melanosome. Note=Identified by mass spectrometry in melanosome fractions from stage I to stage IV.

Tissue Specificity:
Widely expressed; high expression found in skeletal muscle.

DISEASE:
Defects in RAB7A are the cause of Charcot-Marie-Tooth disease type 2B (CMT2B) [MIM:600882]; also known as hereditary motor and sensory neuropathy II (HMSN2). CMT2B is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathy or CMT1, and primary peripheral axonal neuropathy or CMT2. Neuropathies of the CMT2 group are characterized by signs of axonal regeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy. CMT2B is clinically characterized by marked distal muscle weakness and a high frequency of foot ulcers, infections and amputations of the toes. CMT2B inheritance is autosomal dominant.

Similarity:
Belongs to the small GTPase superfamily. Rab family.

Database links:

Entrez Gene: 7879 Human

Entrez Gene: 19349 Mouse

Entrez Gene: 29448 Rat

Omim: 602298 Human

SwissProt: P51149 Human

SwissProt: P51150 Mouse

SwissProt: P09527 Rat

Unigene: 741172 Human

Unigene: 333233 Mouse

Unigene: 486309 Mouse

Unigene: 1425 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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