PMID: 6408304Jan 1, 1983Paper

In-vitro responses to ascorbate and manganese in fibroblasts from a patient with prolidase deficiency and iminodipeptiduria: cell growth, prolidase activity and collagen metabolism

Journal of Inherited Metabolic Disease
I MyaraJ Frey

Abstract

After successful ascorbate and manganese treatment of a female patient with prolidase deficiency and iminodipeptiduria, we attempted to explain the mechanism of action of these drugs in vitro, using them preferentially on skin fibroblasts. Since in vivo, ascorbate and manganese seemed to be responsible for both biochemical and clinical improvement, they were also expected to activate prolidase activity in vitro. Cell growth and prolidase activity were accordingly observed in fibroblast cultures supplemented with these compounds. It seemed that only ascorbate accounted for the successful in vivo response. To understand the mechanism involved, we studied collagen metabolism and found a decreased proline pool, a massive increase of rapidly degraded collagen and moderate enhancement of type III collagen and type I trimer in the patient's fibroblasts. We believe that ascorbate allowed the prolidase-deficient cells to maintain a normal collagen pool by increasing collagen synthesis. Both the massive increase in cell growth in response to ascorbate and the bad response as regards the quality of the collagen produced confirm the secondary nature of this mechanism. However, the relationship between accelerated collagen catabolism and pr...Continue Reading

References

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Citations

Dec 3, 1999·Amino Acids·J LeA Chamson
Jan 1, 1988·Journal of Inherited Metabolic Disease·G MiechA Lemonnier
Jan 1, 1984·Journal of Inherited Metabolic Disease·J Butterworth, D Priestman
Jul 30, 1985·Clinica Chimica Acta; International Journal of Clinical Chemistry·I MyaraA Lemonnier
Dec 1, 1987·Clinica Chimica Acta; International Journal of Clinical Chemistry·I Myara
Apr 29, 1988·Clinica Chimica Acta; International Journal of Clinical Chemistry·H KodamaJ Arata
Mar 15, 1989·Clinica Chimica Acta; International Journal of Clinical Chemistry·H KodamaI Miyoshi
Jan 31, 1990·Clinica Chimica Acta; International Journal of Clinical Chemistry·T OhhashiH Kodama
Oct 15, 1993·Clinica Chimica Acta; International Journal of Clinical Chemistry·I MyaraA Lemonnier
Feb 1, 1994·The International Journal of Biochemistry·I MyaraA Lemonnier
Jul 18, 1997·Journal of Chromatography. B, Biomedical Sciences and Applications·G ZanaboniP Iadarola
Nov 1, 1993·Journal of the American Academy of Dermatology·R BissonnetteR Dubuc
Nov 1, 1984·American Journal of Medical Genetics·B J FreijV M Der Kaloustian
May 12, 2009·Advances in Applied Microbiology·Casey M TheriotAmy M Grunden
Dec 8, 2015·The International Journal of Lower Extremity Wounds·Esra AdışenMehmet Ali Gürer
May 28, 2021·Genetics in Medicine : Official Journal of the American College of Medical Genetics·Francis RossignolManuel Schiff
May 21, 1984·Life Sciences·I MyaraA Lemonnier

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