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Hexb rabbit pAb

Hexb rabbit pAb

ENT-A5694

Description

 

 

 

REF ENT-A5694
Category Antibody Polyclonal
Description Hexb rabbit pAb
Source Rabbit
Applications WB;IHC;IF;ELISA
Reactivity Human;Rat;Mouse;
Reactivity Human;Rat;Mouse;
Dilution Western Blot: 1/500 – 1/2000. Immunohistochemistry: 1/100 – 1/300. Immunofluorescence: 1/200 – 1/1000. ELISA: 1/20000. Not yet tested in other applications.
Immunogen The antiserum was produced against synthesized peptide derived from human HEXB. AA range:481-530
Storage Stability -20°C/1 year
Clonality Polyclonal
Isotype IgG
Concentration 1 mg/ml
Observed Band KD 63kD
Human Gene ID 3074
Human Swiss Prot Nº P07686
Subcellular Location Lysosome . Cytoplasmic vesicle, secretory vesicle, Cortical granule .

Other Name: HEXB; HCC7; Beta-hexosaminidase subunit beta; Beta-N-acetylhexosaminidase subunit beta; Hexosaminidase subunit B; Cervical cancer proto-oncogene 7 protein; HCC-7; N-acetyl-beta-glucosaminidase subunit beta

Background: 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. [provided by RefSeq, May 2014],