HEXA (89-529) Mouse Monoclonal Antibody [Clone ID: AT20F1]

Specifications

Product Data
Clone Name	AT20F1
Applications	ELISA, FACS, FC, WB
Recommend Dilution	ELISA. Western blot: Recommended starting dilution is 1:3000. Flow cytometry.
Reactivity	Human
Host	Mouse
Clonality	Monoclonal
Immunogen	Recombinant human HEXA (89-529aa) purified from E. coli
Specificity	This antibody detects HEXA at aa 89-529.
Isotype	IgG2a, lambda
Formulation	Liquid in. Phosphate-Buffered Saline (pH 7.4) with 0.02% Sodium Azide, 10% glycerol
Concentration	1mg/ml (determined by BCA assay)
Purification	By protein-A affinity chromatography
Conjugation	Unconjugated
Storage Condition	Can be stored at +2°C to +8°C for 1 week. For long term storage, aliquot and store at -20°C to -80C. Avoid repeated freezing and thawing cycles.
Gene Name	hexosaminidase subunit alpha
Database Link	NP_000511.1 Entrez Gene 3073 Human UniProt ID P06865
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.
Synonyms	beta-hexosaminidase subunit alpha, TSD, hexosaminidase A
Reference Data

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