SGSH antibody (AA 301-388)

Catalog No. ABIN1386573

Product Details anti-SGSH Antibody

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

Binding Specificity: AA 301-388

Reactivity: Rat

Host: Rabbit

Clonality: Polyclonal

Conjugate: This SGSH antibody is un-conjugated

Application: Western Blotting (WB), ELISA, Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunocytochemistry (ICC), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))

Cross-Reactivity: Rat

Predicted Reactivity: Human,Mouse,Dog

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human Sulphamidase

Isotype: IgG

Application Details

Application Notes: WB 1:300-5000
ELISA 1:500-1000
IHC-P 1:200-400
IHC-F 1:100-500
IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200
ICC 1:100-500

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 μg/μL

Buffer: 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.

Preservative: ProClin

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

Storage: 4 °C,-20 °C

Storage Comment: Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

Expiry Date: 12 months

Target Details for SGSH

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

Alternative Name: SGSH/Sulphamidase (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:

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.

Gene ID: 6448

UniProt: P51688

Pathways: Glycosaminoglycan Metabolic Process