Hexosaminidase A antibody (AA 89-529)

Catalog No. ABIN5775909

This Mouse Monoclonal antibody specifically detects Hexosaminidase A in WB, ELISA and FACS. It exhibits reactivity toward Human.

Quick Overview for Hexosaminidase A antibody (AA 89-529) (ABIN5775909)

Target: Hexosaminidase A (HEXA)

Reactivity: Human

Host: Mouse

Clonality: Monoclonal

Conjugate: This Hexosaminidase A antibody is un-conjugated

Application: Western Blotting (WB), ELISA, Flow Cytometry (FACS)

Clone: AT20F1

Product Details anti-Hexosaminidase A Antibody

Binding Specificity: AA 89-529

Purpose: Human Hexosaminidase A/HEXA antibody

Purification: Purified

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

Isotype: IgG2a lambda

Application Details

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 mg/mL

Buffer: Phosphate-Buffered Saline ( pH 7.4) with 0.02 % Sodium Azide, 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,-80 °C

Storage Comment: Can be stored at +2C to +8C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.

Target Details for Hexosaminidase A

Target: Hexosaminidase A (HEXA)

Alternative Name: Hexosaminidase A/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.

NCBI Accession: NP_000511

Pathways: Sensory Perception of Sound, Glycosaminoglycan Metabolic Process