Rabbit SGSH Polyclonal Antibody | anti-SGSH antibody

SGSH Rabbit Polyclonal

Cat. Num. AAA7604046

• Gene Names: SGSH; HSS; SFMD; MPS3A
• Reactivity: Human, Mouse, Rat
• Applications: ELISA, Western Blot, Immunohistochemistry
• Purity: >=95% as determined by SDS-PAGE; Immunogen Affinity Purified
• Synonyms: SGSH; Polyclonal Antibody; SGSH Rabbit Polyclonal; anti-SGSH antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: >=95% as determined by SDS-PAGE; Immunogen Affinity Purified
• Form/Format: Liquid
• Sequence Length: 502

• Applicable Applications for anti-SGSH antibody: ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
• Immunogen: N-sulfoglucosamine sulfohydrolase
• Preparation and Storage: PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded human stomach using MBS7604046 (SGSH Antibody) at dilution of 1:50)

Western Blot (WB)

(HEK-293 cells were subjected to SDS PAGE followed by western blot with MBS7604046 (SGSH Antibody) at dilution of 1:1000)

NCBI and Uniprot Product Information

• NCBI GI #: 823672428
• NCBI GeneID: 6448
• Molecular Weight: 56,695 Da
• NCBI Official Full Name: SGSH, partial
• NCBI Official Synonym Full Names: N-sulfoglucosamine sulfohydrolase
• NCBI Official Symbol: SGSH
• NCBI Official Synonym Symbols: HSS; SFMD; MPS3A
• NCBI Protein Information: N-sulphoglucosamine sulphohydrolase
• UniProt Protein Name: N-sulphoglucosamine sulphohydrolase
• Protein Family: N-sulphoglucosamine sulphohydrolase
• UniProt Gene Name: SGSH
• UniProt Synonym Gene Names: HSS
• UniProt Entry Name: SPHM_HUMAN

NCBI Description

This gene encodes one of several enzymes involved in the lysosomal degradation of heparan sulfate. Mutations in this gene are associated with Sanfilippo syndrome A, one type of the lysosomal storage disease mucopolysaccaridosis III, which results from impaired degradation of heparan sulfate. Transcripts of varying sizes have been reported but their biological validity has not been determined. [provided by RefSeq, Jul 2008]

Uniprot Description

SGSH: Defects in SGSH are the cause of mucopolysaccharidosis type 3A (MPS3A); also known as Sanfilippo syndrome A. MPS3A is a severe form of mucopolysaccharidosis type 3, an autosomal recessive lysosomal storage disease due to impaired degradation of heparan sulfate. MPS3 is characterized by severe central nervous system degeneration, but only mild somatic disease. Onset of clinical features usually occurs between 2 and 6 years; severe neurologic degeneration occurs in most patients between 6 and 10 years of age, and death occurs typically during the second or third decade of life. MPS3A is characterized by earlier onset, rapid progression of symptoms and shorter survival. Belongs to the sulfatase family.

Protein type: Hydrolase; Glycan Metabolism - glycosaminoglycan degradation; EC 3.10.1.1

Chromosomal Location of Human Ortholog: 17q25.3

Cellular Component: lysosomal lumen

Molecular Function: catalytic activity; metal ion binding; N-sulfoglucosamine sulfohydrolase activity; sulfuric ester hydrolase activity

Biological Process: glycosaminoglycan catabolic process; proteoglycan metabolic process

Disease: Mucopolysaccharidosis, Type Iiia