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

Specifications

Product Data
Clone Name	AT20F1
Applications	ELISA, 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.
Formulation	PBS, pH 7.4 containing 0.02% Sodium Azide and 10% Glycerol State: Purified State: Liquid purified Ig fraction
Concentration	lot specific
Purification	Protein-A affinity chromatography
Conjugation	Unconjugated
Storage Condition	Store undiluted at 2-8°C for up to two weeks or (in aliquots) at -20°C for longer. Avoid repeated freezing and thawing.
Gene Name	hexosaminidase subunit alpha
Database Link	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-N-acetylhexosaminidase; MGC99608; N-acetyl-beta-glucosaminidase; TSD
Reference Data

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