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Anti-HEXA Antibody

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产品名称: Anti-HEXA Antibody
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简单介绍

Anti-HEXA Antibody


Anti-HEXA Antibody  的详细介绍
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.
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.
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