Hexosaminidase A anticorps
Aperçu rapide pour Hexosaminidase A anticorps (ABIN1846273)
Antigène
Voir toutes Hexosaminidase A (HEXA) AnticorpsReactivité
Hôte
Clonalité
Conjugué
Application
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Purification
- Purified antibody
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Immunogène
- Purified recombinant fragment of human HEXA expressed in E. coli.
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Isotype
- IgG2b
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Indications d'application
- Optimal working dilution should be determined by the investigator.
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Restrictions
- For Research Use only
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Format
- Liquid
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Concentration
- 1.0 mg/mL
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Buffer
- PBS with 0.05 % sodium azide and 0.5 % protein stabilizer.
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Agent conservateur
- Sodium azide
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Précaution d'utilisation
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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Stock
- -20 °C
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Stockage commentaire
- Aliquot and store at -20 °C. Avoid repeated freeze/thaw cycles.
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- Hexosaminidase A (HEXA)
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Autre désignation
- HEXA
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Sujet
- This gene encodes the alpha 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.Alpha subunit gene mutations lead to Tay-Sachs disease (GM2-gangliosidosis type I).
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Poids moléculaire
- 60.7 kDa
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ID gène
- 3073, 15211, 300757
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Pathways
- Sensory Perception of Sound, Glycosaminoglycan Metabolic Process
Antigène
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