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SGSH N-sulfoglucosamine sulfohydrolase [Homo sapiens (human)]

RefSeq Accession Definition Service Stock Status Price *Turnaround time Order
NM_000199 Homo sapiens N-sulfoglucosamine sulfohydrolase (SGSH), mRNA. GenEZ ORF Cloning On-demand $699.00 18
XM_005257582 PREDICTED: Homo sapiens N-sulfoglucosamine sulfohydrolase (SGSH), transcript variant X1, mRNA. GenEZ ORF Cloning On-demand $549.00 14
XM_005257583 PREDICTED: Homo sapiens N-sulfoglucosamine sulfohydrolase (SGSH), transcript variant X2, mRNA. GenEZ ORF Cloning On-demand $549.00 14

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Gene Symbol SGSH
Entrez Gene ID 6448
Full Name N-sulfoglucosamine sulfohydrolase
Synonyms HSS, MPS3A, SFMD
General protein information
Preferred Names
N-sulphoglucosamine sulphohydrolase
N-sulphoglucosamine sulphohydrolase
heparan sulfate sulfatase
sulfoglucosamine sulfamidase
mucopolysaccharidosis type IIIA
Gene Type protein-coding
Organism Homo sapiens (human)



Summary 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].

MIM: 605270

Mucopolysaccharidisis type IIIA (Sanfilippo A), 252900 (3)

mRNA Protein Product Sequence Price Select
NM_000199, 182765452 NP_000190, 4506919 N-sulphoglucosamine sulphohydrolase precursor ORF Sequence $550.00
XM_005257582, 530412677 XP_005257639, 530412678 N-sulphoglucosamine sulphohydrolase isoform X1 ORF Sequence $400.00
XM_005257583, 578831247 XP_005257640, 530412680 N-sulphoglucosamine sulphohydrolase isoform X2 ORF Sequence $400.00
hsa00531Glycosaminoglycan degradation
hsa_M00078Heparan sulfate degradation
REACT_474Metabolism of carbohydrates
REACT_147734MPS II - Hunter syndrome
REACT_147857MPS I - Hurler syndrome
REACT_147749MPS IIID - Sanfilippo syndrome D
REACT_147798MPS IV - Morquio syndrome B
REACT_147825MPS IV - Morquio syndrome A
REACT_147719MPS VI - Maroteaux-Lamy syndrome
REACT_147753MPS IIIA - Sanfilippo syndrome A
REACT_147739MPS IX - Natowicz syndrome
REACT_147860MPS IIIC - Sanfilippo syndrome C
REACT_147788MPS IIIB - Sanfilippo syndrome B
REACT_147759MPS VII - Sly syndrome
REACT_121315Glycosaminoglycan metabolism
REACT_121314Heparan sulfate/heparin (HS-GAG) metabolism
REACT_120752HS-GAG degradation
REACT_200783Myoclonic epilepsy of Lafora
REACT_200833Glycogen storage diseases
Homo sapiens (human)SGSHNP_000190.1
Macaca mulatta (Rhesus monkey)SGSHXP_001110101.2
Canis lupus familiaris (dog)SGSHNP_001003114.1
Bos taurus (cattle)SGSHNP_001095659.2
Mus musculus (house mouse)SgshNP_061292.2
Gallus gallus (chicken)SGSHXP_420084.4
Danio rerio (zebrafish)sgshNP_001116740.1
Drosophila melanogaster (fruit fly)CG14291NP_650760.1
Xenopus (Silurana) tropicalis (western clawed frog)sgshNP_001120065.1
GO:0005975carbohydrate metabolic processTAS
GO:0006027glycosaminoglycan catabolic processTAS
GO:0006029proteoglycan metabolic processTAS
GO:0030203glycosaminoglycan metabolic processTAS
GO:0044281small molecule metabolic processTAS
GO:0043202lysosomal lumenTAS
GO:0070062extracellular vesicular exosomeIDA
GO:0003824catalytic activityTAS
GO:0008484sulfuric ester hydrolase activityIEA
GO:0016250N-sulfoglucosamine sulfohydrolase activityIEA
GO:0046872metal ion bindingIEA
GeneCards SGSH
UniProt P51688
Vega OTTHUMG00000177569
MIM 605270
Ensembl ENSG00000181523
HGNC 10818
HPRD 05590

GeneRIFs: Gene References Into Functions What's a GeneRIF?

What is the normal function of the SGSH gene?

The SGSH gene provides instructions for producing an enzyme called sulfamidase. This enzyme is located in lysosomes, compartments within cells that digest and recycle different types of molecules. Sulfamidase is involved in the step-wise breakdown of large molecules called glycosaminoglycans (GAGs). GAGs are composed of sugar molecules that are linked together to form a long string. To break down these large molecules, individual sugars are removed one at a time from one end of the molecule. Sulfamidase removes a chemical group known as a sulfate from a sugar called glucosamine when it is at the end of the GAG chain.


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