Mouse Anti-ALS2 Recombinant Antibody (4F9) (CBMAB-A2465-YC)

Name: Mouse Anti-ALS2 Recombinant Antibody (4F9)
SKU: CBMAB-A2465-YC
Rating: 5 (1 reviews)

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Datasheet

SDS

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Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

4F9

Application

WB, ELISA

Immunogen

ALS2 (AAH29174, 221 a.a. ~ 320 a.a) 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.

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 221-321

More Infomation

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.

Sprute, R., Jergas, H., Ölmez, A., Alawbathani, S., Karasoy, H., Dafsari, H. S., ... & Cirak, S. (2021). Genotype–phenotype correlation in seven motor neuron disease families with novel ALS2 mutations. American Journal of Medical Genetics Part A, 185(2), 344-354.

Nogueira, E., Alarcón, J., Garma, C., & Paredes, C. (2021). ALS2-related disorders in Spanish children. Neurological Sciences, 42(5), 2091-2094.

Helal, M., Mazaheri, N., Shalbafan, B., Malamiri, R. A., Dilaver, N., Buchert, R., ... & Maroofian, R. (2018). Clinical presentation and natural history of infantile-onset ascending spastic paralysis from three families with an ALS2 founder variant. Neurological Sciences, 39(11), 1917-1925.

Sato, K., Otomo, A., Ueda, M. T., Hiratsuka, Y., Suzuki-Utsunomiya, K., Sugiyama, J., ... & Hadano, S. (2018). Altered oligomeric states in pathogenic ALS2 variants associated with juvenile motor neuron diseases cause loss of ALS2-mediated endosomal function. Journal of Biological Chemistry, 293(44), 17135-17153.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

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

Isotype control

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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