KEGG   Homo sapiens (human): 6448Help
Entry
6448              CDS       T01001                                 

Gene name
SGSH, HSS, MPS3A, SFMD
Definition
(RefSeq) N-sulfoglucosamine sulfohydrolase
  KO
K01565  N-sulfoglucosamine sulfohydrolase [EC:3.10.1.1]
Organism
hsa  Homo sapiens (human)
Pathway
hsa00531  Glycosaminoglycan degradation
hsa01100  Metabolic pathways
hsa04142  Lysosome
Module
hsa_M00078  Heparan sulfate degradation
Disease
H00130  Mucopolysaccharidosis type III (MPS3)
H00421  Mucopolysaccharidosis (MPS)
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)
BRITE hierarchy
SSDB OrthologParalogGFIT
Motif
Pfam: Sulfatase DUF4976 Phosphodiest DUF1501 DUF229
Motif
Other DBs
NCBI-GeneID: 6448
NCBI-ProteinID: NP_000190
OMIM: 605270
HGNC: 10818
Ensembl: ENSG00000181523
Vega: OTTHUMG00000177569
Pharos: P51688(Tbio)
UniProt: P51688
Structure
PDB: 

Jmol
Position
17q25.3
AA seq 502 aa AA seqDB search
MSCPVPACCALLLVLGLCRARPRNALLLLADDGGFESGAYNNSAIATPHLDALARRSLLF
RNAFTSVSSCSPSRASLLTGLPQHQNGMYGLHQDVHHFNSFDKVRSLPLLLSQAGVRTGI
IGKKHVGPETVYPFDFAYTEENGSVLQVGRNITRIKLLVRKFLQTQDDRPFFLYVAFHDP
HRCGHSQPQYGTFCEKFGNGESGMGRIPDWTPQAYDPLDVLVPYFVPNTPAARADLAAQY
TTVGRMDQGVGLVLQELRDAGVLNDTLVIFTSDNGIPFPSGRTNLYWPGTAEPLLVSSPE
HPKRWGQVSEAYVSLLDLTPTILDWFSIPYPSYAIFGSKTIHLTGRSLLPALEAEPLWAT
VFGSQSHHEVTMSYPMRSVQHRHFRLVHNLNFKMPFPIDQDFYVSPTFQDLLNRTTAGQP
TGWYKDLRHYYYRARWELYDRSRDPHETQNLATDPRFAQLLEMLRDQLAKWQWETHDPWV
CAPDGVLEEKLSPQCQPLHNEL
NT seq 1509 nt NT seq  +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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