Hexosaminidase A antibody (AA 89-529)

Catalog No. ABIN5540052

The Mouse Monoclonal anti-Hexosaminidase A antibody has been validated for WB, FACS and EIA. It is suitable to detect Hexosaminidase A in samples from Human.

Quick Overview for Hexosaminidase A antibody (AA 89-529) (ABIN5540052)

Target: Hexosaminidase A (HEXA)

Reactivity: Human

Host: Mouse

Clonality: Monoclonal

Conjugate: This Hexosaminidase A antibody is un-conjugated

Application: Western Blotting (WB), Flow Cytometry (FACS), Enzyme Immunoassay (EIA)

Clone: AT20F1

Product Details anti-Hexosaminidase A Antibody

Binding Specificity: AA 89-529

Specificity: This antibody detects HEXA at aa 89-529.

Purification: Protein-A affinity chromatography

Immunogen: Recombinant human HEXA (89-529aa) purified from E. coli

Isotype: IgG2a

Application Details

Application Notes: ELISA. Western blot: Recommended starting dilution is 1:3000. Flow cytometry.

Restrictions: For Research Use only

Handling

Format: Liquid

Buffer: PBS, pH 7.4 containing 0.02 % Sodium Azide and 10 % Glycerol

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: Store undiluted at 2-8°C for up to two weeks or (in aliquots) at -20°C for longer. Avoid repeated freezing and thawing. Shelf life: one year from despatch.

Expiry Date: 12 months

Target Details for Hexosaminidase A

Target: Hexosaminidase A (HEXA)

Alternative Name: beta-hexosaminidase alpha,hexa

Background: HEXA (Hexosaminidase A), also designated beta-Hexosaminidase A, is responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues. A mutation in the a subunit of hexosaminidase is the cause of Tay-Sachs disease (TSD), also known as GM2-gangliosidosis type I. TSD is a fatal autosomal recessive lysosomal storage disease of the central nervous system (CNS) caused by insufficient activity of the HEXA enzyme that results in a failure to process GM2 gangliosides. The accumulation of GM2 ganglioside in the absence of HEXA activity causes progressive destruction of the CNS.

UniProt: P06865

Pathways: Sensory Perception of Sound, Glycosaminoglycan Metabolic Process