SGSH antibody (AA 301-388) (AbBy Fluor® 350)

Catalog No. ABIN1393137

This anti-SGSH antibody is a Rabbit Polyclonal antibody detecting SGSH in WB, IF (cc) and IF (p). Suitable for Rat.

Quick Overview for SGSH antibody (AA 301-388) (AbBy Fluor® 350) (ABIN1393137)

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

Reactivity: Rat

Host: Rabbit

Clonality: Polyclonal

Conjugate: This SGSH antibody is conjugated to AbBy Fluor® 350

Application: Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Product Details anti-SGSH Antibody (AbBy Fluor® 350)

Binding Specificity: AA 301-388

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: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Restrictions: For Research Use only

Handling

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.

Preservative: ProClin

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

Storage: -20 °C

Storage Comment: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Expiry Date: 12 months

Target Details for SGSH

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

Alternative Name: SGSH/Sulphamidase

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