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LRSAM1 Rabbit pAb (bs-9387R)  
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50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价
产品编号 bs-9387R
英文名称 LRSAM1 Rabbit pAb
中文名称 LRSAM1蛋白抗体
别    名 E3 ubiquitin protein ligase LRSAM1; hTAL; Leucine rich repeat and sterile alpha motif containing protein 1; RIFLE; TAL; Tsg101 associated ligase; LRSM1_HUMAN.  
研究领域 细胞生物  信号转导  细胞粘附分子  
抗体来源 Rabbit
克隆类型 Polyclonal
克 隆 号
交叉反应 Mouse (predicted: Human,Rat,Sheep,Cow,Horse)
产品应用 WB=1:500-2000,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 84 kDa
检测分子量
细胞定位 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human LRSAM1: 201-300/723 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store 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.
PubMed PubMed
产品介绍 LRSAM1 is an E3 ubiquitin-protein ligase that mediates monoubiquitination of TSG101 at multiple sites, leading to inactivation of the ability of TSG101 to sort endocytic (EGF receptors) and exocytic (HIV-1 viral proteins) cargos. It selectively regulates cell adhesion molecules and plays a role in receptor endocytosis and viral budding. LRSAM1 contains a RING-type zinc finger, 5 leucine-rich repeats and 1 SAM (sterile alpha motif) domain. The coiled coil domains interact with the SB domain of TSG101. The PTAP motifs mediate the binding to UEV domains. There are 3 isoforms produced by alternative splicing.

Function:
E3 ubiquitin-protein ligase that mediates monoubiquitination of TSG101 at multiple sites, leading to inactivate the ability of TSG101 to sort endocytic (EGF receptors) and exocytic (HIV-1 viral proteins) cargos.

Subunit:
Interacts with TSG101.

Subcellular Location:
Cytoplasm. Note=Displays a punctuate distribution and localizes to a submembranal ring.

Tissue Specificity:
Highly expressed in adult spinal cord motoneurons as well as in fetal spinal cord and muscle tissue.

DISEASE:
Defects in LRSAM1 are a cause of Charcot-Marie-Tooth disease type 2P (CMT2P) [MIM:614436]. CMT2P is an axonal form of Charcot-Marie-Tooth disease, a disorder of the peripheral nervous system, characterized by progressive weakness and atrophy, initially of the peroneal muscles and later of the distal muscles of the arms. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies (designated CMT1 when they are dominantly inherited) and primary peripheral axonal neuropathies (CMT2). Neuropathies of the CMT2 group are characterized by signs of axonal degeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy. Nerve conduction velocities are normal or slightly reduced.

Similarity:
Contains 6 LRR (leucine-rich) repeats.
Contains 1 RING-type zinc finger.
Contains 1 SAM (sterile alpha motif) domain.

SWISS:
Q6UWE0

Gene ID:
90678

Database links:

Entrez Gene: 90678 Human

Omim: 610933 Human

SwissProt: Q6UWE0 Human

Unigene: 495188 Human



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