Product Name

SGSH antibody

Documents

Description

SGSH Rabbit Polyclonal antibody. Positive WB detected in HEK-293 cells, Transfected HEK-293 cells. Positive IHC detected in human pancreas cancer tissue, human liver cancer tissue.

Tested applications

ELISA, IHC, WB

Species reactivity

Human,Mouse,Rat; other species not tested.

Alternative names

HSS antibody; MPS3A antibody; SFMD antibody; SGSH antibody; Sulfoglucosamine sulfamidase antibody; Sulphamidase antibody

Isotype

Rabbit IgG

Preparation

This antibody was obtained by immunization of SGSH recombinant protein (Accession Number: NM_000199). Purification method: Antigen affinity purified.

Clonality

Polyclonal

Formulation

PBS with 0.02% sodium azide and 50% glycerol pH 7.3.

Storage instructions

Store at -20℃. DO NOT ALIQUOT

Applications

Recommended Dilution:

WB: 1:200-1:2000

IHC: 1:20-1:200

Validations

HEK-293 cells were subjected to SDS PAGE followed by western blot with Catalog No:115173(SGSH Antibody) at dilution of 1:600

Immunohistochemistry of paraffin-embedded human pancreas cancer tissue slide using Catalog No:115173(SGSH Antibody) at dilution of 1:200 (under 10x lens).

Immunohistochemistry of paraffin-embedded human pancreas cancer tissue slide using Catalog No:115173(SGSH Antibody) at dilution of 1:200 (under 40x lens).

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(PMID:21671382). 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.

SGSH antibody

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