Rabbit anti-Human SGSH Polyclonal Antibody | anti-SGSH antibody

SGSH Antibody (C-Term)

Cat. Num. AAA9211715

• Gene Names: SGSH; HSS; SFMD; MPS3A
• Reactivity: Human
• Applications: Western Blot, ELISA
• Purity: Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)
• Synonyms: SGSH; Polyclonal Antibody; SGSH Antibody (C-Term); N-sulphoglucosamine sulphohydrolase; Sulfoglucosamine sulfamidase; Sulphamidase; HSS; anti-SGSH antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: Rabbit Ig
• Specificity: This SGSH antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 420-449 amino acids from the C-terminal region of human SGSH.
• Purity/Purification: Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)
• Form/Format: Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.
• Sequence Positions: 420-449
• Sequence Length: 502

• Applicable Applications for anti-SGSH antibody: Western Blot (WB), ELISA (EIA)
• Application Notes: WB~~1:4000
• Function: Catalyzes a step in lysosomal heparan sulfate degradation.
• Cellular Location: Lysosome.
• Preparation and Storage: Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.

Western Blot (WB)

(Anti-SGSH Antibody (C-Term) at 1:4000 dilution + HepG2 whole cell lysate Lysates/proteins at 20 ug per lane. Secondary Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution. Predicted band size : 57 kDa Blocking/Dilution buffer: 5% NFDM/TBST.)

Related Product Information for anti-SGSH antibody

SGSH is 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.

Product Categories/Family for anti-SGSH antibody

Neuroscience; Signal Transduction

References

Sleat, D.E., et al. Mol. Cell Proteomics 5(4):686-701(2006)
?Bekri, S., et al. J. Inherit. Metab. Dis. 28(4):601-602(2005)
?Muschol, N., et al. Hum. Mutat. 23(6):559-566(2004)
?Zhang, H., et al. Nat. Biotechnol. 21(6):660-666(2003)

NCBI and Uniprot Product Information

• NCBI GI #: 4506919
• NCBI GeneID: 6448
• NCBI Accession #: NP_000190.1
• NCBI GenBank Nucleotide #: NM_000199.3
• UniProt Accession #: P51688; A8K5E2
• NCBI Official Full Name: N-sulphoglucosamine sulphohydrolase
• 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: EC 3.10.1.1; Hydrolase; Glycan Metabolism - glycosaminoglycan degradation

Chromosomal Location of Human Ortholog: 17q25.3

Cellular Component: lysosomal lumen

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

Biological Process: proteoglycan metabolic process; glycosaminoglycan catabolic process; glycosaminoglycan metabolic process; carbohydrate metabolic process; pathogenesis

Disease: Mucopolysaccharidosis, Type Iiia