ALS2 antibody (AA 1384-1440) (Biotin)

Catalog No. ABIN1392877

Product Details anti-ALS2 Antibody (Biotin)

Target: ALS2 (Amyotrophic Lateral Sclerosis 2 (Juvenile) (ALS2))

Binding Specificity: AA 1384-1440

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This ALS2 antibody is conjugated to Biotin

Application: ELISA, Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))

Predicted Reactivity: Human,Mouse,Rat,Dog,Cow,Sheep,Pig,Horse,Rabbit

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human ALS2

Isotype: IgG

Application Details

Application Notes: IHC-P 1:200-400
IHC-F 1:100-500

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Preservative: ProClin

Precaution of Use: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Storage: -20 °C

Storage Comment: Store at -20°C for 12 months.

Expiry Date: 12 months

Target Details for ALS2

Target: ALS2 (Amyotrophic Lateral Sclerosis 2 (Juvenile) (ALS2))

Alternative Name: ALS2/Alsin (ALS2 Products)

Background:

Synonyms: ALS 2, ALS2, ALS2_HUMAN, ALS2CR6, Alsin, ALSJ, Amyotrophic lateral sclerosis 2 juvenile, Amyotrophic lateral sclerosis 2 juvenile chromosome region candidate 6, Amyotrophic lateral sclerosis 2 chromosomal region candidate gene 6 protein, Amyotrophic lateral sclerosis 2 protein, Amyotrophic lateral sclerosis protein 2, FLJ31851, IAHSP, KIAA1563, MGC87187, PLSJ.

Background: Mutations in the ALS2 gene result in a number of juvenile recessive motor neuron diseases (MNDs), including juvenile primary lateral sclerosis (JPLS), a recessive form of amyotrophic lateral sclerosis (ALS2), infantile onset ascending hereditary spastic paralysis (IAHSP), and a form of complicated hereditary spastic paraplegia (cHSP). The ALS2 gene encodes the Alsin protein. Alsin acts as a guanine nucleotide exchange factor for Rab5, a modulator of the endocytic pathway. Alsin is a cytosolic protein that is associated with small, punctate membrane structures. Therefore, Alsin may mediate membrane transport events, potentially linking endocytic processes and actin cytoskeleton remodeling. The ALS2 C-terminal-like protein (ALS2CL) also modulates Rab 5 activity.

Pathways: Skeletal Muscle Fiber Development