GAA antibody (N-Term)

Catalog No. ABIN656475

Product Details anti-GAA Antibody

Target: GAA (Glucosidase, Alpha, Acid (GAA))

Binding Specificity: AA 174-203, N-Term

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This GAA antibody is un-conjugated

Application: Western Blotting (WB), Immunohistochemistry (IHC), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))

Purification: This antibody is purified through a protein A column, followed by peptide affinity purification.

Immunogen: This GAA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 174-203 amino acids from the N-terminal region of human GAA.

Clone: RB18979

Isotype: IgG

Application Details

Application Notes: WB: 1:2000. WB: 1:1000. WB: 1:1000. WB: 1:1000. IHC-P-Leica: 1:500. IHC-P-Leica: 1:500

Restrictions: For Research Use only

Handling

Format: Liquid

Buffer: Purified polyclonal antibody supplied in PBS with 0.09 % (W/V) sodium azide.

Preservative: Sodium azide

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

Storage: 4 °C,-20 °C

Storage Comment: GAA Antibody (N-term) can be refrigerated at 2-8 °C for up to 6 months. For long term storage, place the at -20 °C.

Expiry Date: 6 months

Target Details for GAA

Target: GAA (Glucosidase, Alpha, Acid (GAA))

Alternative Name: GAA (GAA Products)

Synonyms: LYAG antibody, E430018M07Rik antibody, glucosidase alpha, acid antibody, glucosidase, alpha, acid antibody, transmembrane and coiled-coil domain family 1 antibody, glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II) antibody, GAA antibody, Gaa antibody, TMCC1 antibody, gaa antibody

Background: This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. Three transcript variants encoding the same protein have been found for this gene.

Molecular Weight: 105324

Gene ID: 2548

NCBI Accession: NP_000143, NP_001073271, NP_001073272

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process