SGSH Antibody - Cat. #: CSB-PA021200ESR1HU

SKU: CSB-PA021200ESR1HU

Size :50ul

Clone Number:

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

Product Type:Polyclonal Antibody

Immunogen Species:Homo sapiens (Human)

UniProt ID:P51688

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

Raised in:Rabbit

Species Reactivity:Human

Tested Applications:ELISA, IHC; Recommended dilution: IHC:1:20-1:200

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.

Clonality:Polyclonal

Isotype:IgG

Purification Method:Antigen Affinity Purified

Conjugate:Non-conjugated

Buffer:PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Form:Liquid

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

Target Names:SGSH

Research Areas:Neuroscience; Tags & Cell Markers; Signal transduction