SGSH antibody (AA 273-502)

Catalog No. ABIN7160782

Product Details anti-SGSH Antibody

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

Binding Specificity: AA 273-502

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This SGSH antibody is un-conjugated

Application: Immunohistochemistry (IHC), ELISA

Cross-Reactivity: Human

Purification: Antigen Affinity Purified

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

Isotype: IgG

Application Details

Application Notes: Recommended dilution: IHC:1:20-1:200,

Restrictions: For Research Use only

Handling

Format: Liquid

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

Preservative: Sodium azide

Precaution of Use: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Storage: -20 °C,-80 °C

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

Target Details for SGSH

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

Alternative Name: SGSH (SGSH Products)

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

Background:

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