Rabbit Polyclonal Antibody to HEXA

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别名:TSD
应用:WB,IHC
反应种属:Human, Mouse, Rat
规格：50μl/100μl

Description
This gene encodes a member of the glycosyl hydrolase 20 family of proteins. The encoded preproprotein is proteolytically processed to generate the alpha subunit of the lysosomal enzyme beta-hexosaminidase. This enzyme, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Mutations in this gene lead to an accumulation of GM2 ganglioside in neurons, the underlying cause of neurodegenerative disorders termed the GM2 gangliosidoses, including Tay-Sachs disease (GM2-gangliosidosis type I). Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed.

Description

This gene encodes a member of the glycosyl hydrolase 20 family of proteins. The encoded preproprotein is proteolytically processed to generate the alpha subunit of the lysosomal enzyme beta-hexosaminidase. This enzyme, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Mutations in this gene lead to an accumulation of GM2 ganglioside in neurons, the underlying cause of neurodegenerative disorders termed the GM2 gangliosidoses, including Tay-Sachs disease (GM2-gangliosidosis type I). Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed.

Specification
Aliases	TSD
Swissprot	P06865
WB Predicted band size	61 kDa
Host/Isotype	Rabbit IgG
Storage	Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Species Reactivity	Human, Mouse, Rat
Immunogen	Fusion protein of human HEXA
Formulation	pH7.4 PBS, 0.05% NaN3, 40% Glycerol

Application
WB	1/500-1/2000
IHC	1/100-1/200
ELISA	1/5000-1/10000

	Gel: 8%SDS-PAGE, Lysate: 40 μg, Lane 1-3: Jurkat, PC3 and HT-29 cell lysates, Primary antibody: P03444(HEXA Antibody) at dilution 1/700, Secondary antibody: Goat anti rabbit IgG at 1/5000 dilution, Exposure time: 1 minute
	The image is immunohistochemistry of paraffin-embedded Human tonsil tissue using P03444(HEXA Antibody) at dilution 1/100. (Original magnification: ×200)
	The image is immunohistochemistry of paraffin-embedded Human liver cancer tissue using P03444(HEXA Antibody) at dilution 1/100. (Original magnification: ×200)