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.
Specificity
Anti-ALS2 (N-terminal region) specifically recognizes human, rat, and mouse ALS2.
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).
Physical form
Solution in 0.01 M phosÂphate 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, 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.
- UPC:
- 12352203
- Condition:
- New
- Availability:
- 3-5 Days
- Weight:
- 1.00 Ounces
- HazmatClass:
- No
- WeightUOM:
- LB
- MPN:
- SAB4200137-200UL
- Product Size:
- 200/µL