Alsin (ALS2) Antibody
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Description
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 nucleotide exchange factor for the small GTPase RAB5. The protein localizes with RAB5 on early endosomal compartments, and functions as a modulator for endosomal dynamics. Mutations in its gene result in several forms of juvenile lateral sclerosis and infantile-onset ascending spastic paralysis.
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Product specifications
| Category | Primary Antibodies |
| Immunogen Target | Alsin (ALS2) |
| Host | Rabbit |
| Reactivity | Human, Mouse |
| Recommended Dilution | WB: 1/1000, IHC-P: 1/50 - 1/100. Not tested in IHC-F. Optimal dilutions/concentrations should be determined by the end user. |
| Clonality | Polyclonal |
| Conjugation | Unconjugated |
| Isotype | IgG |
| Purification | Purified Rabbit Polyclonal Antibody. |
| Size 1 | 80 µl |
| Size 2 | 400 µl |
| Form | Liquid |
| Tested Applications | ELISA, WB, IHC |
| Buffer | PBS containing 0.09% sodium azide. |
| Availability | Shipped within 5-10 working days. |
| Storage | Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles. |
| Dry Ice | No |
| UniProt ID | Q96Q42 |
| Alias | ALS2,ALS2CR6 |
| Background | Antibody anti-ALS2 |
| Status | RUO |
Descripción
ALS2, also known as Alsin, refers to a gene that encodes a protein called Alsin. Mutations in the ALS2 gene are associated with a rare autosomal recessive form of amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, a progressive neurodegenerative disorder characterized by the degeneration of motor neurons in the brain and spinal cord. The ALS2 gene provides instructions for producing the Alsin protein, which is involved in various cellular functions, including the regulation of endosome trafficking, maintenance of the cytoskeleton, and protection against oxidative stress. Alsin is particularly important for the health and function of motor neurons, the nerve cells that control voluntary muscle movement. Mutations in the ALS2 gene can lead to dysfunctional or truncated forms of the Alsin protein, which can disrupt normal cellular processes and contribute to the degeneration of motor neurons seen in ALS. However, it's worth noting that ALS2 mutations are relatively rare and account for only a small percentage of ALS cases.
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Alsin (ALS2) Antibody
ALS2 Antibody is a Rabbit Polyclonal antibody against ALS2. The protein encoded by this gene 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 nucleotide exchange factor for the small GTPase RAB5. The protein localizes with RAB5 on early endosomal compartments, and functions as a modulator for endosomal dynamics. Mutations in this gene result in several forms of juvenile lateral sclerosis and infantile-onset ascending spastic paralysis. Multiple transcript variants encoding different isoforms have been found for this gene.
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Alsin (ALS2) Antibody
ALS2 Antibody is a Rabbit Polyclonal antibody against ALS2. The protein encoded by this gene 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 nucleotide exchange factor for the small GTPase RAB5. The protein localizes with RAB5 on early endosomal compartments, and functions as a modulator for endosomal dynamics. Mutations in this gene result in several forms of juvenile lateral sclerosis and infantile-onset ascending spastic paralysis. Multiple transcript variants encoding different isoforms have been found for this gene.
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