Hexosaminidase A anticorps

N° du produit ABIN7256120

Détail du produit anti-Hexosaminidase A anticorps

Antigène: Hexosaminidase A (HEXA)

Reactivité: Humain, Souris, Rat

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp Hexosaminidase A est non-conjugé

Application: Immunohistochemistry (IHC)

Attributs du produit: Polyclonal Antibody

Purification: Affinity purification

Immunogène: Recombinant fusion protein of human HEXA (NP_000511.2).

Isotype: IgG

Information d'application

Indications d'application: IHC 1:50-1:200

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 1 mg/mL

Buffer: PBS with 0.02 % sodium azide, 50 % glycerol, pH 7.3

Agent conservateur: Sodium azide

Précaution d'utilisation: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Stock: -20 °C

Stockage commentaire: Store at -20°C. Avoid freeze / thaw cycles.

Détails sur Hexosaminidase A

Antigène: Hexosaminidase A (HEXA)

Autre désignation: HEXA (HEXA Produits)

Synonymes: anticorps TSD, anticorps fc04h11, anticorps wu:fc04h11, anticorps wu:fv09f06, anticorps si:dkey-35i22.2, anticorps HEXA, anticorps Hex-1, anticorps zgc:112084, anticorps hexosaminidase subunit alpha, anticorps hexosaminidase B (beta polypeptide), anticorps CUGBP Elav-like family member 6, anticorps hexosaminidase A, anticorps hexosaminidase A (alpha polypeptide), anticorps beta-hexosaminidase subunit alpha-like, anticorps HEXA, anticorps hexb, anticorps CELF6, anticorps Hexa, anticorps hexa, anticorps LOC100228061

Sujet: This gene encodes a member of the glycosyl hydrolase 20 family of proteins. The encoded preproprotein is proteolytically processed to generate the alpha subunit of the lysosomal enzyme beta-hexosaminidase. This enzyme, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Mutations in this gene lead to an accumulation of GM2 ganglioside in neurons, the underlying cause of neurodegenerative disorders termed the GM2 gangliosidoses, including Tay-Sachs disease (GM2-gangliosidosis type I). Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed.

ID gène: 3073

UniProt: P06865

Pathways: Sensory Perception of Sound, Glycosaminoglycan Metabolic Process