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Mouse Anti-ALS2 Recombinant Antibody (4F10) (CBMAB-A2463-YC)

Provided herein is a Mouse monoclonal antibody against Human ALS2, Alsin Rho Guanine Nucleotide Exchange Factor. The antibody can be used for immunoassay techniques, such as ELISA, IF, WB.
See all ALS2 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
4F10
Antibody Isotype
IgG1, κ
Application
ELISA, IF, WB

Basic Information

Immunogen
ALS2 (AAH29174, 221-320 aa) partial recombinant protein with GST tag.
Host Species
Mouse
Specificity
Human
Antibody Isotype
IgG1, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
IF(ICC)40 µg/ml

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS, pH 7.4
Preservative
None
Concentration
Batch dependent
Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.
Epitope
aa 221-320

Target

Full Name
amyotrophic lateral sclerosis 2 (juvenile)
Introduction
ALS2 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 nucle
Entrez Gene ID
57679
UniProt ID
Q96Q42
Alternative Names
ALS2, Alsin Rho Guanine Nucleotide Exchange Factor; Amyotrophic Lateral Sclerosis 2 Chromosomal Region Candidate Gene 6 Protein; Amyotrophic Lateral Sclerosis 2 (Juvenile); Amyotrophic Lateral Sclerosis 2 Protein; ALS2CR6; Alsin;
Function
May act as a GTPase regulator. Controls survival and growth of spinal motoneurons (By similarity).
Biological Process
Endosome organization Source: MGI
Lysosomal transport Source: MGI
Neuron projection morphogenesis Source: UniProtKB
Positive regulation of GTPase activity Source: UniProtKB
Positive regulation of protein kinase activity Source: UniProtKB
Positive regulation of Rac protein signal transduction Source: UniProtKB
Protein homooligomerization Source: MGI
Regulation of endosome size Source: UniProtKB
Cellular Location
Centrosome; Cytosol; Early endosome; Nucleus; Dendrite; Growth cone; Lamellipodium; Protein-containing complex; Ruffle; Vesicle
Involvement in disease
Amyotrophic lateral sclerosis 2 (ALS2): A neurodegenerative disorder affecting upper motor neurons in the brain and lower motor neurons in the brain stem and spinal cord, resulting in fatal paralysis. Sensory abnormalities are absent. The pathologic hallmarks of the disease include pallor of the corticospinal tract due to loss of motor neurons, presence of ubiquitin-positive inclusions within surviving motor neurons, and deposition of pathologic aggregates. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of the cases.
Juvenile primary lateral sclerosis (JPLS): A neurodegenerative disorder which is closely related to but clinically distinct from amyotrophic lateral sclerosis. It is a progressive paralytic disorder which results from dysfunction of the upper motor neurons while the lower neurons are unaffected.
Infantile-onset ascending spastic paralysis (IAHSP): Characterized by progressive spasticity and weakness of limbs.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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