ALS2 (Alsin, Amyotrophic Lateral Sclerosis Protein 2, Amyotrophic Lateral Sclerosis 2 Chromosomal Region Candidate Gene 6 Protein, KIAA1563, ALS2CR6)

ALS2 contains an ATS1/RCC1-like domain, a RhoGEF domain, and a vacuolar protein sorting 9 (VPS9) domain, all of which are guanine-nucleotide exchange factors that activate members of the Ras superfamily of GTPases. The protein functions as a guanine nucleotide exchange factor for the small GTPase RAB5. The protein localizes with RAB5 on early endosomal compartments, and functions as a modulator for endosomal dynamics. Mutations in its gene result in several forms of juvenile lateral sclerosis and infantile-onset ascending spastic paralysis.\n\nApplications:\nSuitable for use in Immunohistochemistry. Other applications not tested.\n\nRecommended Dilution:\nImmunohistochemistry (paraffin): 3-5ug/ml\nOptimal dilutions to be determined by the researcher.\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

A1362-85C

Size

100ug

Applications

IHC

Hosts

Goat

Reactivities

Hum

Form

Supplied as a liquid in Tris saline, 0.02% sodium azide, pH7.3, 0.5% BSA.

P Type

Pab

Purity

Purified by immunoaffinity chromatography.

Isotype

IgG

References

1. Yang Y, Hentati A, Deng HX, Dabbagh O, Sasaki T, Hirano M, Hung WY, Ouahchi K, Yan J, Azim AC, Cole N, Gascon G, Yagmour A, Ben-Hamida M, Pericak-Vance M, Hentati F, Siddique T. The gene encoding alsin, a protein with three guanine-nucleotide exchange factor domains, is mutated in a form of recessive amyotrophic lateral sclerosis. Nat Genet. 2001 Oct;29(2):160-5. PMID: 11586297.

Additional Info

Recognizes human ALS2. Species sequence homology: mouse and rat.