Linked Life Data

7351504

Source:http://linkedlifedata.com/resource/pubmed/id/7351504

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
2
pubmed:dateCreated
1980-3-27
pubmed:abstractText
Dermatosparaxis, a genetic disease, results from the deficiency of the NH2 procollagen peptidase, an enzyme which removes the NH2-terminal nontriple-helical extensions from procollagen. We have identified a Himalayan cat which has deficient amino terminal procollagen peptidase activity. The partially processed precursor chains pNalpha 1 (110,000 daltons) and pNalpha 2 (99,000 daltons) were identified by sodium dodecyl sulfate electrophoresis. In contrast to that from a normal animal, the 20,000 xg supernatant of a skin homogenate failed to convert pNcollagen to collagen. Amino acid analysis of pNalpha 1 and pNalapha 2 chains demonstrated the presence of cysteine and a lower percentage of hydroxyprolyl and glycyl residues due to the presence of the amino terminal extensions. The disorder in this animal is milder than that in sheep and cattle which is reflected in the longer survival and relatively smaller proportion of pNalpha chains in skin. The defect was also demonstrated by skin fibroblasts in culture.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Feb
pubmed:issn
0022-202X
pubmed:author
pubmed:issnType
Print
pubmed:volume
74
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
96-9
pubmed:dateRevised
2006-11-15
pubmed:meshHeading
pubmed:year
1980
pubmed:articleTitle
Dermatosparaxis in a Himalayan cat: I. Biochemical studies of dermal collagen.
pubmed:publicationType
Journal Article, Research Support, U.S. Gov't, P.H.S.