SAB4200136
Anti-ALS2 antibody produced in rabbit
~1.5 mg/mL, affinity isolated antibody
Synonym(s):
Anti-ALS2CR6, Anti-ALSJ, Anti-Alsin, Anti-IAHSP, Anti-PLSJ
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About This Item
UNSPSC Code:
12352203
NACRES:
NA.41
biological source
rabbit
conjugate
unconjugated
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
Learn more about Antibody Enhanced Validation
concentration
~1.5 mg/mL
technique(s)
western blot: 1.5-3.0 μg/mL using HEK-293T cell lysates expressing human ALS2
UniProt accession no.
shipped in
dry ice
Storage temp.
−20°C
target post-translational modification
unmodified
Gene Information
human ... ALS2(57679)
rat ... Als2(363235)
General description
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.
Application
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.
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Storage Class
10 - Combustible liquids
flash_point_f
Not applicable
flash_point_c
Not applicable
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Shinji Hadano et al.
Human molecular genetics, 15(2), 233-250 (2005-12-03)
ALS2/alsin is a member of guanine nucleotide exchange factors for the small GTPase Rab5 (Rab5GEFs), which act as modulators in endocytic pathway. Loss-of-function mutations in human ALS2 account for a number of juvenile recessive motor neuron diseases (MNDs). However, the
Huaibin Cai et al.
Neuro-degenerative diseases, 5(6), 359-366 (2008-08-21)
Autosomal recessive mutations in the ALS2 gene have been linked to juvenile-onset amyotrophic lateral sclerosis (ALS2), primary lateral sclerosis and juvenile-onset ascending hereditary spastic paraplegia. Except for two recently identified missense mutations, all other mutations in the ALS2 gene lead
Shakeela Daud et al.
Amyotrophic lateral sclerosis & frontotemporal degeneration, 17(3-4), 260-265 (2016-01-12)
Biallelic mutations of ALS2 cause a clinical spectrum of overlapping autosomal recessive neurodegenerative disorders: infantile-onset ascending hereditary spastic paralysis (IAHSP), juvenile primary lateral sclerosis (JPLS), and juvenile amyotrophic lateral sclerosis (ALS2). We report on eleven individuals affected with IAHSP from
Francois Gros-Louis et al.
Human molecular genetics, 17(17), 2691-2702 (2008-06-19)
Recessive ALS2 mutations are linked to three related but slightly different neurodegenerative disorders: amyotrophic lateral sclerosis, hereditary spastic paraplegia and primary lateral sclerosis. To investigate the function of the ALS2 encoded protein, we generated Als2 knock-out (KO) mice and zAls2
Ryota Kunita et al.
The Journal of biological chemistry, 282(22), 16599-16611 (2007-04-06)
Mutations in the ALS2 gene cause a number of recessive motor neuron diseases, indicating that the ALS2 protein (ALS2/alsin) is vital for motor neurons. ALS2 acts as a guanine nucleotide exchange factor (GEF) for Rab5 (Rab5GEF) and is involved in
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