Linked Life Data

10224661

Source:http://linkedlifedata.com/resource/pubmed/id/10224661

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
3-4
pubmed:dateCreated
1999-6-8
pubmed:abstractText
Sulphamidase is one of four lysosomal proteins whose deficiency clinically manifests as Sanfilippo syndrome. Deficiency of sulphamidase results in the lysosomal storage of the glycosaminoglycan (GAG) heparan sulphate (HS) and is termed mucopolysaccharidosis type IIIA (MPS IIIA). Sulphamidase catalyses the hydrolysis of an N-linked sulphate from the nonreducing terminal glucosaminide residue of HS (Fig. 1). It is unique among the known lysosomal sulphatases involved in GAG degradation in that it is an N-sulphatase, all the others being O-sulphatases. Purification of sulphamidase from human liver has facilitated the amino-terminal sequencing of the protein and hence the isolation of cDNA and genomic clones for sulphamidase. This has in turn made possible a range of further studies aimed at better diagnosis, treatment and understanding of MPS IIIA.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:issn
1357-2725
pubmed:author
pubmed:issnType
Print
pubmed:volume
31
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
363-7
pubmed:dateRevised
2008-8-16
pubmed:meshHeading
pubmed:articleTitle
Sulphamidase.
pubmed:affiliation
Department of Chemical Pathology, Women's and Children's Hospital, North Adelaide, Australia. danson@medicine.adelaide.edu.au
pubmed:publicationType
Journal Article, Review