Goat anti-Human Alsin Polyclonal Antibody | anti-ALS2 antibody

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

Cat. Num. AAA649300

• Gene Names: ALS2; ALSJ; PLSJ; IAHSP; ALS2CR6
• Reactivity: Human
• Applications: ELISA, Immunohistochemistry
• Purity: Affinity Purified; Purified by immunoaffinity chromatography.
• Synonyms: Alsin; Polyclonal Antibody; CT (ALS2; Amyotrophic Lateral Sclerosis 2 Chromosomal Region Candidate Gene 6 Protein; Amyotrophic Lateral Sclerosis 2 Protein; ALS2CR6; KIAA1563); Anti -Alsin; anti-ALS2 antibody

• Host: Goat
• Reactivity: Human
• Clonality: Polyclonal
• Specificity: Recognizes human ALS2. Species sequence homology: Canine, mouse and rat.
• Purity/Purification: Affinity Purified; Purified by immunoaffinity chromatography.
• Form/Format: Supplied as a liquid in TBS, pH 7.3, 0.5% BSA, 0.02% sodium azide.

• Applicable Applications for anti-ALS2 antibody: ELISA (EL/EIA), Immunohistochemistry (IHC)
• Application Notes: Suitable for use in ELISA and Immunohistochemistry.; Dilution: ELISA: 1:8000; Immunohistochemistry (Formalin-fixed, paraffin-embedded): 3.75ug/ml
• Immunogen: Synthetic peptide corresponding to LKACYYQIQREKLN from the C-terminal of human ALS2.
• Preparation and Storage: 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.

Immunohistochemistry (IHC)

(Human Brain, Cortex (formalin-fixed, paraffin-embedded) stained with ALS2 antibody at 3.75ug/ml followed by biotinylated goat IgG secondary antibody alkaline phosphatase-streptavidin and chromogen.)

Related Product Information for anti-ALS2 antibody

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.

Product Categories/Family for anti-ALS2 antibody

Antibodies; Abs to Disease Markers

NCBI and Uniprot Product Information

• NCBI GI #: 209364523
• NCBI GeneID: 57679
• NCBI Accession #: NP_001129217.1
• NCBI GenBank Nucleotide #: NM_001135745.1
• UniProt Accession #: Q96Q42; Q53TT1; Q53TV2; Q8N1E0; Q96PC4; Q96Q41; Q9H973; Q9HCK9
• Molecular Weight: 183,634 Da
• NCBI Official Full Name: alsin isoform 2
• NCBI Official Synonym Full Names: amyotrophic lateral sclerosis 2 (juvenile)
• NCBI Official Symbol: ALS2
• NCBI Official Synonym Symbols: ALSJ; PLSJ; IAHSP; ALS2CR6
• NCBI Protein Information: alsin; amyotrophic lateral sclerosis 2 protein; amyotrophic lateral sclerosis 2 chromosomal region candidate gene 6 protein
• UniProt Protein Name: Alsin
• Protein Family: Alsin
• UniProt Gene Name: ALS2
• UniProt Synonym Gene Names: ALS2CR6; KIAA1563
• UniProt Entry Name: ALS2_HUMAN

NCBI Description

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]

Uniprot Description

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.