Anti-ALS2 antibody produced in rabbit

NACRES:

NA.41

Кат. номер

SAB4200136-25UL

SAB4200136-200UL

Amyotrophic lateral sclerosis 2 (ALS2) protein also called as alsin, consists of three putative guanine exchange factor (GEF) domains, the RCC1-like domain (RLD), the Dbl and pleckstrin homology (DH/PH) domain, and a vacuolar protein sorting 9 (VPS9) domain. In addition, this protein also contains eight consecutive membrane occupation and recognition nexus (MORN) motifs. The ALS2 mRNA is ubiquitously expressed in the central nervous system (CNS) and non-neuronal tissues, with the highest expression in the cerebellum and kidney. The gene ALS2 is mapped to human chromosome 2q33.1.
Specificity
Anti-ALS2 specifically recognizes human ALS2.
Anti-ALS2 antibody produced in rabbit is suitable for immunoblotting.
Biochem/physiol Actions
Loss-of-function mutations in the amyotrophic lateral sclerosis (ALS2) gene, accounts for several recessive motor neuron diseases, including forms of ALS , juvenile-onset PLS (PLSJ) and hereditary spastic paraplegia (HSP). ALS is a heterogeneous neurological disorder that affects large motor neurons of the central nervous system (CNS). ALS2 protein contains several domains, implicating roles in cell signaling and membrane dynamics. This protein has been shown to mediate the activation of Ras-related protein Rab-5A (Rab5) and Ras-related C3 botulinum toxin substrate 1 (Rac1)/ p21-activated kinase (PAK1). In the nervous system, the protein ALS2 is preferentially associated with the cytoplasmic side of the endosome membrane, it modulates endosome membrane trafficking and promotes neurite growth in neuronal cultures. ALS2 gene knockout mice exhibits neurological deficits, altered endosome trafficking, and degeneration of corticospinal neurons.
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, 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.

biological source	rabbit
Quality Level	200
antibody form	affinity isolated antibody
antibody product type	primary antibodies
clone	polyclonal
form	buffered aqueous solution
mol wt	antigen ~185 kDa
species reactivity	human
packaging	antibody small pack of 25 µL
enhanced validation	recombinant expression
concentration	~1.5 mg/mL
application(s)	western blot: 1.5-3.0 µg/mL using HEK-293T cell lysates expressing human ALS2
conjugate	unconjugated
UniProt accession no.	Q96Q42,
shipped in	dry ice
storage temp.	20°C
Gene Information	human ... ALS2(57679) rat ... Als2(363235)

RIDADR	NONH for all modes of transport
Flash Point F	Not applicable
Flash Point C	Not applicable