SGSH anticorps (AA 273-502)

N° du produit ABIN7160782

Détail du produit anti-SGSH anticorps

Antigène: SGSH (N-Sulfoglucosamine Sulfohydrolase (SGSH))

Épitope: AA 273-502

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp SGSH est non-conjugé

Application: Immunohistochemistry (IHC), ELISA

Réactivité croisée: Humain

Purification: Antigen Affinity Purified

Immunogène: Recombinant Human N-sulphoglucosamine sulphohydrolase protein (273-502AA)

Isotype: IgG

Information d'application

Indications d'application: Recommended dilution: IHC:1:20-1:200,

Restrictions: For Research Use only

Stockage

Format: Liquid

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,-80 °C

Stockage commentaire: Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Détails sur SGSH

Antigène: SGSH (N-Sulfoglucosamine Sulfohydrolase (SGSH))

Autre désignation: SGSH (SGSH Produits)

Synonymes: anticorps HSS, anticorps MPS3A, anticorps SFMD, anticorps 4632406A19Rik, anticorps N-sulfoglucosamine sulfohydrolase, anticorps N-sulfoglucosamine sulfohydrolase (sulfamidase), anticorps SGSH, anticorps Sgsh

Sujet:

Background: SGSH(N-sulphoglucosamine sulphohydrolase) is also named as HSS, sulphamidase and belongs to the sulfatase family. Sulfamidase is synthesized as a 62 kDa precursor protein, which is modified with mannose 6-phosphate (M6P) residues, allowing their recognition by mannose-6-phosphate receptors in the Golgi complex and ensuring transport to the endosomal/lysosomal system. It catalyzes the third step of degradation of glucosaminoglycans and is required for the degradation of heparan sulphate. Defects in SGSH are the cause of mucopolysaccharidosis type 3A (MPS3A), also known as Sanfilippo syndrome A.

Aliases: Heparan sulfate sulfatase antibody, Heparan sulphate sulphatase antibody, HSS antibody, MPS 3A antibody, MPS3 A antibody, MPS3A antibody, Mucopolysaccharidosis type IIIA antibody, N sulfoglucosamine sulfohydrolase (sulfamidase) antibody, N sulfoglucosamine sulfohydrolase antibody, N-sulphoglucosamine sulphohydrolase antibody, SFMD antibody, SGSH antibody, SPHM_HUMAN antibody, Sulfoglucosamine sulfamidase antibody, Sulphamidase antibody, Sulphoglucosamine sulphamidase antibody

UniProt: P51688

Pathways: Glycosaminoglycan Metabolic Process