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Merck
CN

SAB4200137

Sigma-Aldrich

Anti-ALS2 (N-terminal region) antibody produced in rabbit

~1.5 mg/mL, affinity isolated antibody

别名:

Anti-ALS2CR6, Anti-ALSIN, Anti-ALSJ, Anti-IAHSP, Anti-PLSJ

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关于此项目

UNSPSC代码:
12352203
NACRES:
NA.41
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生物来源

rabbit

偶联物

unconjugated

抗体形式

affinity isolated antibody

抗体产品类型

primary antibodies

克隆

polyclonal

表单

buffered aqueous solution

分子量

antigen ~185 kDa

包装

antibody small pack of 25 μL

浓度

~1.5 mg/mL

技术

indirect immunofluorescence: 2-4 μg/mL using NIH3T3 cells
western blot: 1.5-3.0 μg/mL using HEK-293T cell lysate over expressing human ALS2, and rat cerebellum extract (S1 fraction).

运输

dry ice

储存温度

−20°C

靶向翻译后修饰

unmodified

基因信息

mouse ... Als2(74018)
rat ... Als2(363235)

相关类别

一般描述

Amyotrophic lateral sclerosis (ALS)2 gene codes for 184 kDa alsin protein. Alsin is characterized with the three putative guanine exchange factor (GEF) domains such as, RCC1-like domain (RLD), the Dbl and pleckstrin homology (DH/PH) domain, and a vacuolar protein sorting 9 (VPS9) domain. In addition, ALS2 also contains eight consecutive membrane occupation and recognition nexus (MORN) motifs. ALS2 mRNA is ubiquitously expressed in the CNS and non-neuronal tissues, with the highest expression in the cerebellum and kidney.

应用

Anti-ALS2 (N-terminal region) antibody produced in rabbit has been used in immunoblotting and immunoprecipitation.

生化/生理作用

Amyotrophic lateral sclerosis (ALS)2 has been shown to mediate the activation of Rab5 and Rac1/PACK1. In the nervous system, ALS2 is preferentially associated with the cytoplasmic side of endosome membrane, it modulates endosome membrane trafficking and promotes neurite growth in neuronal cultures. Loss-of function mutations in the ALS2 gene leads to the development of recessive motor neuron diseases, including forms of ALS (ALS2), juvenile PLS (PLSJ) and hereditary spastic paraplegia (HSP).
Anti-ALS2 (N-terminal region) specifically recognizes human, rat, and mouse ALS2.

外形

Solution in 0.01 M phos­phate buffered saline, pH 7.4, containing 15 mM sodium azide.

制备说明

For continuous use, store at 2–8 °C for up to one month. For extended storage, freeze in working aliquots. Repeated freezing and thawing, or storage in “frost-free” freezers, is not recommended. If slight turbidity occurs upon prolonged storage, clarify the solution by centrifugation before use. Working dilutions should be discarded if not used within 12 hours.

免责声明

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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储存分类代码

10 - Combustible liquids

闪点(°F)

Not applicable

闪点(°C)

Not applicable

法规信息

常规特殊物品

历史批次信息供参考:

分析证书(COA)

Lot/Batch Number

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Mice deficient in the Rab5 guanine nucleotide exchange factor ALS2/alsin exhibit age-dependent neurological deficits and altered endosome trafficking
Hadano S, et al.
Human Molecular Genetics, 15, 233-250 (2005)
Gönenç Çobanoğlu et al.
Biochemical and biophysical research communications, 427(1), 1-4 (2012-09-18)
Mutations in ALS2 gene/alsin are associated with recessive forms of motor neuron disorders including Juvenile Amyotrophic Lateral Sclerosis (JALS), Infantile-onset Ascending Hereditary Spastic Paraplegia (IAHSP) and Juvenile Primary Lateral Sclerosis (JPLS). In this study, we show that alsin and another
The Rab5 Activator ALS2/alsin Acts as a Novel Rac1 Effector through Rac1-activated Endocytosis
Kunita R, et al.
The Journal of Biological Chemistry, 282, 16599-16611 (2007)
Alsin and the Molecular Pathways of Amyotrophic Lateral Sclerosis
Chandran J, et al.
Molecular Neurobiology, 36, 224-224 (2007)
Izzet Enunlu et al.
Biochemical and biophysical research communications, 413(3), 471-475 (2011-09-13)
Mutations in Als2 gene cause several autosomal recessive forms of motor neuron diseases including Juvenile Amyotrophic Lateral Sclerosis (JALS), Juvenile Primary Lateral Sclerosis (PLSJ) and Infantile-onset Ascending Hereditary Spastic Paralysis (IAHSP). To find novel protein-protein interactions of Als2 protein we

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