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Normal Production, Nature, and Extent of Intracellular Degradation of Newly Synthesized Collagen in Fibroblasts from a Patient with Prolidase Deficiency
- Source :
- Connective Tissue Research. 29:23-30
- Publication Year :
- 1993
- Publisher :
- Informa UK Limited, 1993.
-
Abstract
- 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.
- Subjects :
- Dipeptidases
medicine.medical_specialty
Proline
Biology
Biochemistry
Pathogenesis
Rheumatology
Internal medicine
medicine
Humans
Orthopedics and Sports Medicine
Carbon Radioisotopes
Fibroblast
Molecular Biology
Cells, Cultured
chemistry.chemical_classification
Prolidase deficiency
Cell Biology
Metabolism
Fibroblasts
medicine.disease
In vitro
Hydroxyproline
Endocrinology
medicine.anatomical_structure
Enzyme
chemistry
Collagen
Intracellular
Subjects
Details
- ISSN :
- 16078438 and 03008207
- Volume :
- 29
- Database :
- OpenAIRE
- Journal :
- Connective Tissue Research
- Accession number :
- edsair.doi.dedup.....f16a31a4ba9ddc0e10871d5a235e9c8d