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Anti-HEXA Antibody

品牌：Antibodies

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规格：50µl

货期：

应用：

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Anti-HEXA Antibody

商品详情参考文献相关资料

Overview

Name:	Anti-HEXA Antibody See all HEXA primary antibodies
Description:	Rabbit polyclonal antibody to HEXA.
Applications:	WB
Dilutions:	WB: 1:500 - 1:2000.
Reactivity:	Human, Mouse
Immunogen:	Recombinant protein of human HEXA.
Protein Length:	529
Host:	Rabbit
Clonality:	Polyclonal
Isotype:	IgG
Conjugate:	Unconjugated
Purification:	Affinity purification.
Product Form:	Liquid
Formulation:	Supplied in Phosphate Buffered Saline, pH 7.30, with 0.02% Sodium Azide and 50% Glycerol.
Storage:	Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.

Target

Function:	Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues. The form B is active against certain oligosaccharides. The form S has no measurable activity.
Involvement in Disease:	GM2-gangliosidosis 1: An autosomal recessive lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. It is characterized by GM2 gangliosides accumulation in the absence of HEXA activity, leading to neurodegeneration and, in the infantile form, death in early childhood. It exists in several forms: infantile (most common and most severe), juvenile and ***** (late-onset).
Sequence Similarities:	Belongs to the glycosyl hydrolase 20 family.
Post-Translational Modification:	N-linked glycan at Asn-115 consists of Man(3)-GlcNAc(2).
Cellular Location:	Lysosome.
Database Links:	Entrez Gene: 3073 Human Entrez Gene: 15211 Mouse Omim: 606869 Human SwissProt: P06865 Human SwissProt: P29416 Mouse Unigene: 604479 Human Unigene: 709495 Human Unigene: 2284 Mouse
Synonyms:	Beta hexosaminidase alpha chain precursor Antibody Beta hexosaminidase subunit alpha Antibody Beta N acetylhexosaminidase Antibody Beta N acetylhexosaminidase subunit alpha Antibody Beta-hexosaminidase A Antibody Beta-hexosaminidase subunit alpha Antibody Beta-N-acetylhexosaminidase subunit alpha Antibody HEXA Antibody HEXA_HUMAN Antibody Hexosaminidase A Antibody Hexosaminidase A (alpha polypeptide) Antibody Hexosaminidase A alpha polypeptide Antibody Hexosaminidase subunit A Antibody MGC99608 Antibody N acetyl beta glucosaminidase Antibody N acetyl beta glucosaminidase subunit alpha Antibody N-acetyl-beta-glucosaminidase subunit alpha Antibody TSD Antibody
Information:	Target information shown above is from the UniProt Consortium.