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Protein Page:
SGSH (human)
p Phosphorylation
ac Acetylation
me Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
ub Ubiquitination
sm Sumoylation
ne Neddylation
gl O-GlcNAc
ga O-GalNAc
pa Palmitoylation
ad Adenylylation
sn S-Nitrosylation
ca Caspase cleavage
sc Succinylation

Overview
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. Note: This description may include information from UniProtKB.
Protein type: Glycan Metabolism - glycosaminoglycan degradation; EC 3.10.1.1; Hydrolase
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
Reference #:  P51688 (UniProtKB)
Alt. Names/Synonyms: heparan sulfate sulfatase; HSS; MPS3A; N-sulfoglucosamine sulfohydrolase; N-sulphoglucosamine sulphohydrolase; SFMD; SGSH; SPHM; Sulfoglucosamine sulfamidase; Sulphamidase
Gene Symbols: SGSH
Molecular weight: 56,695 Da
Basal Isoelectric point: 6.46  Predict pI for various phosphorylation states
Select Structure to View Below

SGSH

Protein Structure Not Found.


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Modification Sites and Domains Show Modification Legend
Click here to view phosphorylation modifications only

Modification Sites in Parent Protein, Orthologs, and Isoforms Show Modification Legend
 

Show Multiple Sequence Alignment


 SS 

SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


 MS 

MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


       human

 
0 1 T65 LLFRNAFTSVSSCSP
0 1 S66 LFRNAFTSVSSCSPS
0 1 K103-ub HHFNSFDkVRSLPLL
0 1 Y174-p DDRPFFLyVAFHDPH
0 1 K303-ub VSSPEHPkRWGQVSE
0 1 Y330-p LDWFSIPyPsYAIFG
0 1 S332-p WFSIPyPsYAIFGSK
0 1 T407-p QDFYVSPtFQDLLNR
0 1 K425-ub GQPTGWYkDLRHYYY
  mouse

 
T65-p LIFRNAFtsVSSCSP
S66-p IFRNAFtsVSSCSPS
K103 HHFNSFDKVQSLPLL
Y174 DDRPFFLYVAFHDPH
Q303 VSSPEHPQRWGQVSD
Y330 LDWFSIPYPSYAIFG
S332 WFSIPYPSYAIFGSK
T407 QDFYVSPTFQDLLNR
K425 GRPTGWYKDLHRYYY
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