Technical Data
A1362-85A
ALS2 (ALSIN, amyotrophic lateral sclerosis 2 (juvenile), ALSJ, PLSJ, IAHSP, KIAA1563)
Description:
The protein encoded by this gene 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 this gene result in several forms of juvenile lateral sclerosis and infantile-onset ascending spastic paralysis. Multiple transcript variants encoding different isoforms have been found for this gene.

Applications:
Suitable for use in ELISA. Other applications not tested.

Recommended Dilution:
ELISA: 1:8000
Optimal dilutions to be determined by the researcher.

Storage and Stability:
May be stored at 4C for short-term only. For long-term storage and to avoid repeated freezing and thawing, aliquot and store at -20C. Aliquots are stable for at least 12 months at -20C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
TypeIsotypeCloneGrade
PabAffinity Purified
SizeStorageShippingSourceHost
100ug-20CBlue IceHumanGoat
Concentration:
As reported
Immunogen:
Synthetic peptide of ALS2.
Purity:
Purified by immunoaffinity chromatography.
Form
Supplied as a liquid in 0.5 mg/ml tris-saline, 0.02% sodium azide, pH 7.3, 0.5% BSA.
Specificity:
Recognizes ALS2. Species Crossreactivity: Canine, mouse and rat.
Intended for research use only. Not for use in human, therapeutic, or diagnostic applications.
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.