
Immunohistochemistry of paraffin-embedded human kidney tissue using CSB-PA021200ESR1HU at dilution of 1:100
SGSH Antibody
CSB-PA021200ESR1HU
ApplicationsELISA, ImmunoHistoChemistry
Product group Antibodies
ReactivityHuman
TargetSGSH
Overview
- SupplierCusabio
- Product NameSGSH Antibody
- Delivery Days Customer20
- ApplicationsELISA, ImmunoHistoChemistry
- CertificationResearch Use Only
- ClonalityPolyclonal
- ConjugateUnconjugated
- Gene ID6448
- Target nameSGSH
- Target descriptionN-sulfoglucosamine sulfohydrolase
- Target synonymsheparan sulfate sulfatase; HSS; MPS3A; mucopolysaccharidosis type IIIA; N-sulphoglucosamine sulphohydrolase; SFMD; sulfoglucosamine sulfamidase; sulphamidase
- HostRabbit
- IsotypeIgG
- Protein IDP51688
- Protein NameN-sulphoglucosamine sulphohydrolase
- Scientific DescriptionSGSH(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.
- ReactivityHuman
- Storage Instruction-20°C or -80°C
- UNSPSC12352203