SGSH anticorps (AA 301-388) (Biotin)

N° du produit ABIN1393141

Cet anticorps Lapin Polyclonal détecte spécifiquement SGSH dans WB, ELISA, IHC (p) et IHC (fro). Il présente une réactivité envers Rat.

Aperçu rapide pour SGSH anticorps (AA 301-388) (Biotin) (ABIN1393141)

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

Reactivité: Rat

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp SGSH est conjugé à/à la Biotin

Application: Western Blotting (WB), ELISA, Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))

Détail du produit anti-SGSH anticorps (Biotin)

Épitope: AA 301-388

Réactivité croisée: Rat

Homologie: Human,Mouse,Dog

Purification: Purified by Protein A.

Immunogène: KLH conjugated synthetic peptide derived from human Sulphamidase

Isotype: IgG

Information d'application

Indications d'application: WB 1:300-5000
IHC-P 1:200-400
IHC-F 1:100-500

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Agent conservateur: ProClin

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

Stock: -20 °C

Stockage commentaire: Store at -20°C for 12 months.

Date de péremption: 12 months

Détails sur SGSH

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

Autre désignation: SGSH/Sulphamidase

Sujet:

Synonyms: HSS, SFMD, MPS3A, N-sulphoglucosamine sulphohydrolase, Sulfoglucosamine sulfamidase, Sulphamidase, SGSH

Background: Sulfatases are enzymes that hydrolyse a diverse range of sulfate esters. Deficiency of lysosomal sulfatases leads to human diseases characterized by the accumulation of either GAGs (glycosaminoglycans) or sulfolipids. Sulfamidase, also known as HSS, SFMD, MPS3A or SGSH, is a 502 amino acid lysosome that belongs to the sulfatase family. It has been suggested that sulfamidase may be involved in the lysosomal degradation of heparan sulfate. Defects in the gene encoding sulfamidase are the cause of Sanfilippo syndrome A, an autosomal recessive lysosomal storage disease caused by impaired degradation of heparan sulfate. Sanfilippo syndrome A is characterized by severe central nervous system degeneration but relatively mild somatic manifestations.

ID gène: 6448

UniProt: P51688

Pathways: Glycosaminoglycan Metabolic Process