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Merck
CN

SAB4200137

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

NACRES:
NA.41
UNSPSC Code:
12352203
Conjugate:
unconjugated
Clone:
polyclonal
Application:
IF, WB
Citations:
6
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biological source

rabbit

conjugate

unconjugated

antibody form

affinity isolated antibody

antibody product type

primary antibodies

clone

polyclonal

form

buffered aqueous solution

mol wt

antigen ~185 kDa

packaging

antibody small pack of 25 μL

concentration

~1.5 mg/mL

technique(s)

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).

shipped in

dry ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

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

General description

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.

Application

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

Biochem/physiol Actions

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.

Physical form

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

Preparation Note

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.

Disclaimer

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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存储类别

10 - Combustible liquids

flash_point_f

Not applicable

flash_point_c

Not applicable

法规信息

常规特殊物品
低风险生物材料
此项目有

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)
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
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)

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