Anti -Alsin, CT (ALS2, Amyotrophic Lateral Sclerosis 2 Chromosomal Region Candidate Gene 6 Protein, Amyotrophic Lateral Sclerosis 2 Protein, ALS2CR6, KIAA1563)

For Research Use Only. Not for use in diagnostic procedures.

Chromosome: 2; NC_000002.11 (202564986..202645895, complement). Location: 2q33.1

Polyclonal

Goat

Recognizes human ALS2. Species sequence homology: Canine, mouse and rat.

Affinity Purified
Purified by immunoaffinity chromatography.

Supplied as a liquid in TBS, pH 7.3, 0.5% BSA, 0.02% sodium azide.

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Small volumes of anti-ALS2 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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.

Antibodies; Abs to Disease Markers

ELISA (EL/EIA), Immunohistochemistry (IHC)

Suitable for use in ELISA and Immunohistochemistry.
Dilution: ELISA: 1:8000
Immunohistochemistry (Formalin-fixed, paraffin-embedded): 3.75ug/ml

183,634 Da[Similar Products]

amyotrophic lateral sclerosis 2 (juvenile)

alsin; amyotrophic lateral sclerosis 2 protein; amyotrophic lateral sclerosis 2 chromosomal region candidate gene 6 protein

Alsin

ALS2CR6; KIAA1563??[Similar Products]

ALS2_HUMAN

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. [provided by RefSeq, Oct 2008]

Function: May act as a GTPase regulator. Controls survival and growth of spinal motoneurons

By similarity.

Subunit structure: Forms a heteromeric complex with ALS2CL. Interacts with ALS2CL. Ref.9

Involvement in disease: Amyotrophic lateral sclerosis 2 (ALS2) [MIM:205100]: 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.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.2 Ref.9Juvenile primary lateral sclerosis (JPLS) [MIM:606353]: 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.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.1Infantile-onset ascending spastic paralysis (IAHSP) [MIM:607225]: Characterized by progressive spasticity and weakness of limbs.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.10

Sequence similarities: Contains 1 DH (DBL-homology) domain.Contains 8 MORN repeats.Contains 1 PH domain.Contains 5 RCC1 repeats.Contains 1 VPS9 domain.

Sequence caution: The sequence BAB13389.2 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.