Rabbit Polyclonal Antibody to HEXB

包装: 1G|100G|500G|1KG|100KG

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别名:ENC-1AS; HEL-248; HEL-S-111
应用:WB,IHC
反应种属:Human
规格：50μl/100μl

Description
Hexosaminidase B is the beta subunit of the lysosomal enzyme beta-hexosaminidase that, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Beta-hexosaminidase is composed of two subunits, alpha and beta, which are encoded by separate genes. Both beta-hexosaminidase alpha and beta subunits are members of family 20 of glycosyl hydrolases. Mutations in the alpha or beta subunit genes lead to an accumulation of GM2 ganglioside in neurons and neurodegenerative disorders termed the GM2 gangliosidoses. Beta subunit gene mutations lead to Sandhoff disease (GM2-gangliosidosis type II). Alternatively spliced transcript variants encoding different isoforms have been found for this gene.

Description

Hexosaminidase B is the beta subunit of the lysosomal enzyme beta-hexosaminidase that, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Beta-hexosaminidase is composed of two subunits, alpha and beta, which are encoded by separate genes. Both beta-hexosaminidase alpha and beta subunits are members of family 20 of glycosyl hydrolases. Mutations in the alpha or beta subunit genes lead to an accumulation of GM2 ganglioside in neurons and neurodegenerative disorders termed the GM2 gangliosidoses. Beta subunit gene mutations lead to Sandhoff disease (GM2-gangliosidosis type II). Alternatively spliced transcript variants encoding different isoforms have been found for this gene.

Specification
Aliases	ENC-1AS; HEL-248; HEL-S-111
Swissprot	P07686
WB Predicted band size	63 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
Immunogen	Synthetic peptide of human HEXB
Formulation	pH7.4 PBS, 0.05% NaN3, 40% Glycerol

Application
WB	1/500-1/2000
IHC	1/50-1/100
ELISA	1/5000-1/10000

	Gel: 8%SDS-PAGE, Lysate: 40 μg, Lane: A172 cell lysate, Primary antibody: P13781(HEXB Antibody) at dilution 1/400, Secondary antibody: Goat anti rabbit IgG at 1/5000 dilution, Exposure time: 3 seconds
	The image is immunohistochemistry of paraffin-embedded Human tonsil tissue using P13781(HEXB Antibody) at dilution 1/50. (Original magnification: ×200)
	The image is immunohistochemistry of paraffin-embedded Human cervical cancer tissue using P13781(HEXB Antibody) at dilution 1/50. (Original magnification: ×200)