| Entry |
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| Gene name |
SGSH, HSS, MPS3A, SFMD
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| Definition |
(RefSeq) N-sulfoglucosamine sulfohydrolase
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| KO |
| N-sulfoglucosamine sulfohydrolase [EC: 3.10.1.1] |
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| Organism |
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| Pathway |
| Glycosaminoglycan degradation | | Metabolic pathways | | Lysosome |
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| Module |
| Heparan sulfate degradation |
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| Disease |
| Mucopolysaccharidosis type III (MPS3) | | Mucopolysaccharidosis (MPS) |
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| Brite |
KEGG Orthology (KO) [BR:hsa00001]
Metabolism
Glycan biosynthesis and metabolism
00531 Glycosaminoglycan degradation
6448 (SGSH)
Cellular Processes
Transport and catabolism
04142 Lysosome
6448 (SGSH)
Enzymes [BR:hsa01000]
3. Hydrolases
3.10 Acting on sulfur-nitrogen bonds
3.10.1 Acting on sulfur-nitrogen bonds (only sub-subclass identified to date)
3.10.1.1 N-sulfoglucosamine sulfohydrolase
6448 (SGSH)
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| SSDB |
   |
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| Motif |
 |
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| Other DBs |
NCBI-GeneID: NCBI-ProteinID: OMIM: HGNC: Ensembl: Vega: Pharos: UniProt: |
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| LinkDB |
 |
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| Structure |
PDB: |
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| Position |
17q25.3
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| AA seq |
502 aa  
MSCPVPACCALLLVLGLCRARPRNALLLLADDGGFESGAYNNSAIATPHLDALARRSLLF
RNAFTSVSSCSPSRASLLTGLPQHQNGMYGLHQDVHHFNSFDKVRSLPLLLSQAGVRTGI
IGKKHVGPETVYPFDFAYTEENGSVLQVGRNITRIKLLVRKFLQTQDDRPFFLYVAFHDP
HRCGHSQPQYGTFCEKFGNGESGMGRIPDWTPQAYDPLDVLVPYFVPNTPAARADLAAQY
TTVGRMDQGVGLVLQELRDAGVLNDTLVIFTSDNGIPFPSGRTNLYWPGTAEPLLVSSPE
HPKRWGQVSEAYVSLLDLTPTILDWFSIPYPSYAIFGSKTIHLTGRSLLPALEAEPLWAT
VFGSQSHHEVTMSYPMRSVQHRHFRLVHNLNFKMPFPIDQDFYVSPTFQDLLNRTTAGQP
TGWYKDLRHYYYRARWELYDRSRDPHETQNLATDPRFAQLLEMLRDQLAKWQWETHDPWV
CAPDGVLEEKLSPQCQPLHNEL |
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| NT seq |
1509 nt  +upstreamnt +downstreamnt
atgagctgccccgtgcccgcctgctgcgcgctgctgctagtcctggggctctgccgggcg
cgtccccggaacgcactgctgctcctcgcggatgacggaggctttgagagtggcgcgtac
aacaacagcgccatcgccaccccgcacctggacgccttggcccgccgcagcctcctcttt
cgcaatgccttcacctcggtcagcagctgctctcccagccgcgccagcctcctcactggc
ctgccccagcatcagaatgggatgtacgggctgcaccaggacgtgcaccacttcaactcc
ttcgacaaggtgcggagcctgccgctgctgctcagccaagctggtgtgcgcacaggcatc
atcgggaagaagcacgtggggccggagaccgtgtacccgtttgactttgcgtacacggag
gagaatggctccgtcctccaggtggggcggaacatcactagaattaagctgctcgtccgg
aaattcctgcagactcaggatgaccggcctttcttcctctacgtcgccttccacgacccc
caccgctgtgggcactcccagccccagtacggaaccttctgtgagaagtttggcaacgga
gagagcggcatgggtcgtatcccagactggaccccccaggcctacgacccactggacgtg
ctggtgccttacttcgtccccaacaccccggcagcccgagccgacctggccgctcagtac
accaccgtcggccgcatggaccaaggagttggactggtgctccaggagctgcgtgacgcc
ggtgtcctgaacgacacactggtgatcttcacgtccgacaacgggatccccttccccagc
ggcaggaccaacctgtactggccgggcactgctgaacccttactggtgtcatccccggag
cacccaaaacgctggggccaagtcagcgaggcctacgtgagcctcctagacctcacgccc
accatcttggattggttctcgatcccgtaccccagctacgccatctttggctcgaagacc
atccacctcactggccggtccctcctgccggcgctggaggccgagcccctctgggccacc
gtctttggcagccagagccaccacgaggtcaccatgtcctaccccatgcgctccgtgcag
caccggcacttccgcctcgtgcacaacctcaacttcaagatgccctttcccatcgaccag
gacttctacgtctcacccaccttccaggacctcctgaaccgcaccacagctggtcagccc
acgggctggtacaaggacctccgtcattactactaccgggcgcgctgggagctctacgac
cggagccgggacccccacgagacccagaacctggccaccgacccgcgctttgctcagctt
ctggagatgcttcgggaccagctggccaagtggcagtgggagacccacgacccctgggtg
tgcgcccccgacggcgtcctggaggagaagctctctccccagtgccagcccctccacaat
gagctgtga |
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