Literature DB >> 6762213

Cystine accumulation in cystinotic fibroblasts from free and protein-linked cystine but not cysteine.

J G Thoene, R M Lemons.   

Abstract

The accumulation of cystine in cystinotic fibroblasts from free and protein-linked cystine has been investigated. Cystine is not accumulated from cysteine but is readily accumulated from cystine. The accumulation from free cystine does not occur as a result of pinocytosis or from the degradation of a rapidly metabolized protein pool. Further studies of the degradation of disulphide-containing proteins by these cells may aid understanding of the mechanisms of proteolysis.

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Year:  1982        PMID： 6762213      PMCID： PMC1154037          DOI： 10.1042/bj2080823

Source DB:  PubMed          Journal:  Biochem J        ISSN： 0264-6021            Impact factor:   3.857


  15 in total

1.  The degradative metabolism of L-cysteine and L-cystine in vitro by liver in cystinosis.

Authors:  A D PATRICK
Journal:  Biochem J       Date:  1962-05       Impact factor: 3.857

2.  Cystinosis. Intracellular cystine depletion by aminothiols in vitro and in vivo.

Authors:  J G Thoene; R G Oshima; J C Crawhall; D L Olson; J A Schneider
Journal:  J Clin Invest       Date:  1976-07       Impact factor: 14.808

3.  Cystine metabolism in human fibroblasts. Comparison of normal, cystinotic, and gamma-glutamylcysteine synethetase-deficient cells.

Authors:  R G Oshima; W J Rhead; J G Thoene; J A Schneider
Journal:  J Biol Chem       Date:  1976-07-25       Impact factor: 5.157

4.  Cystinotic fibroblasts accumulate cystine from intracellular protein degradation.

Authors:  J G Thoene; R G Oshima; D G Ritchie; J A Schneider
Journal:  Proc Natl Acad Sci U S A       Date:  1977-10       Impact factor: 11.205

5.  Uptake of cystine by cystine-depleted fibroblasts from patients with cystinosis.

Authors:  B States; J Lee; S Segal
Journal:  Biochem Biophys Res Commun       Date:  1981-01-15       Impact factor: 3.575

6.  Modulation of the intracellular cystine content of cystinotic fibroblasts by extracellular albumin.

Authors:  J G Thoene; R Lemons
Journal:  Pediatr Res       Date:  1980-06       Impact factor: 3.756

7.  Cystine depletion of cystinotic tissues by phosphocysteamine (WR638).

Authors:  J G Thoene; R Lemons
Journal:  J Pediatr       Date:  1980-06       Impact factor: 4.406

8.  Cystine: compartmentalization within lysosomes in cystinotic leukocytes.

Authors:  J D Schulman; K H Bradley; J E Seegmiller
Journal:  Science       Date:  1969-11-28       Impact factor: 47.728

9.  Lysomotropic amines cause intracellular accumulation of receptors for epidermal growth factor.

Authors:  A C King; L Hernaez-Davis; P Cuatrecasas
Journal:  Proc Natl Acad Sci U S A       Date:  1980-06       Impact factor: 11.205

10.  Pinocytosis in fibroblasts. Quantitative studies in vitro.

Authors:  R M Steinman; J M Silver; Z A Cohn
Journal:  J Cell Biol       Date:  1974-12       Impact factor: 10.539
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  9 in total

Review 1.  The renal Fanconi syndrome in cystinosis: pathogenic insights and therapeutic perspectives.

Authors:  Stephanie Cherqui; Pierre J Courtoy
Journal:  Nat Rev Nephrol       Date:  2016-12-19       Impact factor: 28.314

2.  Time course of pathogenic and adaptation mechanisms in cystinotic mouse kidneys.

Authors:  Héloïse P Gaide Chevronnay; Virginie Janssens; Patrick Van Der Smissen; Francisca N'Kuli; Nathalie Nevo; Yves Guiot; Elena Levtchenko; Etienne Marbaix; Christophe E Pierreux; Stéphanie Cherqui; Corinne Antignac; Pierre J Courtoy
Journal:  J Am Soc Nephrol       Date:  2014-02-13       Impact factor: 10.121

3.  Inhibitors of protein synthesis also inhibit lysosomal proteolysis. Studies using cystinotic fibroblasts.

Authors:  J G Thoene; R Lemons; S Boskovich; K Borysko
Journal:  J Clin Invest       Date:  1985-02       Impact factor: 14.808

4.  Protection of Cystinotic Mice by Kidney-Specific Megalin Ablation Supports an Endocytosis-Based Mechanism for Nephropathic Cystinosis Progression.

Authors:  Virginie Janssens; Héloïse P Gaide Chevronnay; Sandrine Marie; Marie-Françoise Vincent; Patrick Van Der Smissen; Nathalie Nevo; Seppo Vainio; Rikke Nielsen; Erik I Christensen; François Jouret; Corinne Antignac; Christophe E Pierreux; Pierre J Courtoy
Journal:  J Am Soc Nephrol       Date:  2019-09-23       Impact factor: 10.121

5.  Role of thiols, pH and cathepsin D in the lysosomal catabolism of serum albumin.

Authors:  J L Mego
Journal:  Biochem J       Date:  1984-03-15       Impact factor: 3.857

6.  Disulphide reduction in lysosomes. The role of cysteine.

Authors:  J B Lloyd
Journal:  Biochem J       Date:  1986-07-01       Impact factor: 3.857

7.  A cysteine-specific lysosomal transport system provides a major route for the delivery of thiol to human fibroblast lysosomes: possible role in supporting lysosomal proteolysis.

Authors:  R L Pisoni; T L Acker; K M Lisowski; R M Lemons; J G Thoene
Journal:  J Cell Biol       Date:  1990-02       Impact factor: 10.539

Review 8.  In Vitro and In Vivo Models to Study Nephropathic Cystinosis.

Authors:  Pang Yuk Cheung; Patrick T Harrison; Alan J Davidson; Jennifer A Hollywood
Journal:  Cells       Date:  2021-12-21       Impact factor: 6.600

9.  Patients With Infantile Nephropathic Cystinosis in Germany and Austria: A Retrospective Cohort Study.

Authors:  Nina O'Connell; Jun Oh; Klaus Arbeiter; Anja Büscher; Dieter Haffner; Jessica Kaufeld; Christine Kurschat; Christoph Mache; Dominik Müller; Ludwig Patzer; Lutz T Weber; Burkhard Tönshoff; Marcus Weitz; Katharina Hohenfellner; Lars Pape
Journal:  Front Med (Lausanne)       Date:  2022-04-25
  9 in total

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