Linked Life Data

8339543

Source:http://linkedlifedata.com/resource/pubmed/id/8339543

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
1
pubmed:dateCreated
1993-9-1
pubmed:abstractText
We have examined the extent of intracellular degradation of newly synthesized collagen occurring in fibroblasts from a patient with prolidase deficiency, a rare, autosomal recessively inherited disorder, in which a lack of prolidase, which normally cleaves imidodipeptides with a C-terminal Pro or Hyp residue, results in hyperimidodipeptiduria. The main clinical feature of the condition is chronic, intractable ulceration of the skin, and the suggestion has been made that it represents a specific disorder of collagen metabolism. Although most of the hydroxy-[14]proline derived from the intracellular degradation of newly synthesized collagen in prolidase-deficient fibroblasts occurred in imidodipeptides, with a similar chromatographic profile to those occurring in the patient's urine, the proportion of collagen undergoing such degradation was as in control cells. No abnormality was found in other parameters of collagen metabolism studied, and the results confirm that, although the pathogenesis of its clinical manifestations remains unclear, the disorder is one of protein degradation in general.
pubmed:grant
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:issn
0300-8207
pubmed:author
pubmed:issnType
Print
pubmed:volume
29
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
23-30
pubmed:dateRevised
2007-11-14
pubmed:meshHeading
pubmed:year
1993
pubmed:articleTitle
Normal production, nature, and extent of intracellular degradation of newly synthesized collagen in fibroblasts from a patient with prolidase deficiency.
pubmed:affiliation
Hollister Laboratories for Connective Tissue Diseases, Meyer Rehabilitation Institute, University of Nebraska Medical Center, Omaha 68198-5430.
pubmed:publicationType
Journal Article, Research Support, U.S. Gov't, P.H.S., Research Support, Non-U.S. Gov't