The amyotrophic lateral sclerosis 2 (ALS2) C-terminal like (ALS2CL) gene is an ALS2 homologous gene, encoding a homo-dimeric protein. The ALS2CL protein forms an ALS2-ALS2CL heteromeric complex with ALS2 oligomer. This complex has been shown to co-localize in membrane compartments. The ALS2CL is mapped to human chromosome 3p21.31.
Specificity
Anti-ALS2CL specifically recognizes human ALS2CL.
Application
Anti-ALS2CL antibody produced in rabbit is suitable for immunoblotting.
Biochem/physiol Actions
The amyotrophic lateral sclerosis 2 (ALS2) C-terminal like (ALS2CL) protein exhibits a weak Ras-related protein Rab-5A (Rab5)-guanine nucleotide exchange factor (GEF) activity, but strongly binds to Rab5. The co-expression of ALS2CL and Rab5 causes the distinctive tubulation phenotype of endosome compartments. It also results in the co-localization of ALS2CL and Rab5. Also, the ALS2CL protein suppresses the endosome enlargement induced by ALS2, resulting in an extensive perinuclear tubulo-membrane phenotype. In contrast to ALS2, ALS2CL might be considered as a critical moiety in modulating the ALS2 and Rab5-mediated endosome dynamics in the cell.
Physical form
Solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15 mM sodium azide.
Storage and Stability
For continuous use, store at 2-8 °C for up to one month. For extended storage, freezeat -20°C, 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.
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ALS2, the causative gene product for juvenile recessive amyotrophic lateral sclerosis (ALS2), is a guanine-nucleotide exchange factor for the small GTPase Rab5. Here, we report a novel ALS2 homologous gene, ALS2 C-terminal like (ALS2CL), which encodes a 108-kD ALS2CL protein.
Biochemical and biophysical research communications, 354(2), 491-497 (2007-01-24)
ALS2, the causative gene product for a number of recessive motor neuron diseases, is a guanine-nucleotide exchange factor for Rab5, and acts as a modulator for endosome dynamics. Recently, we have identified a novel ALS2 homolog, ALS2CL, which is highly
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