Literature DB >> 3663103

Cystine storage in cultured myotubes from patients with nephropathic cystinosis.

G S Harper1, I Bernardini, O Hurko, J Zuurveld, W A Gahl.   

Abstract

Sorted muscle cells, cultured from a patient with nephropathic cystinosis, stored 100 times normal amounts of cystine. Subcellular fractionation and density-gradient centrifugation confirmed that the cystine was located in a lysosomal compartment. 2. Myoblasts from cystinotic patients in culture underwent fusion to myotubes in a normal fashion. 3. The free thiol cysteamine effectively depleted cystinotic-muscle cells of cystine. 4. Cultured myoblast and myotubes offered a unique system for investigating the effects of lysosomal storage on differentiated cell functions.

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Year:  1987        PMID： 3663103      PMCID： PMC1147933          DOI： 10.1042/bj2430841

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


  20 in total

1.  Protein measurement with the Folin phenol reagent.

Authors:  O H LOWRY; N J ROSEBROUGH; A L FARR; R J RANDALL
Journal:  J Biol Chem       Date:  1951-11       Impact factor: 5.157

2.  A quantitative procedure for the dissociation of adult mammalian muscle into mononucleated cells.

Authors:  R Yasin; G van Beers; D Bulien; E J Thompson
Journal:  Exp Cell Res       Date:  1976-10-15       Impact factor: 3.905

3.  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

4.  A quantitative technique for growing human adult skeletal muscle in culture starting from mononucleated cells.

Authors:  R Yasin; G Van Beers; K C Nurse; S Al-Ani; D N Landon; E J Thompson
Journal:  J Neurol Sci       Date:  1977-07       Impact factor: 3.181

5.  Two species of lysosomal organelles in cultured human fibroblasts.

Authors:  L H Rome; A J Garvin; M M Allietta; E F Neufeld
Journal:  Cell       Date:  1979-05       Impact factor: 41.582

6.  Biochemical comparisons of the adult and childhood forms of cystinosis.

Authors:  J A Schneider; V Wong; K Bradley; J E Seegmiller
Journal:  N Engl J Med       Date:  1968-12-05       Impact factor: 91.245

7.  Surface antigen differentiation during human myogenesis in culture.

Authors:  F S Walsh; M A Ritter
Journal:  Nature       Date:  1981-01-01       Impact factor: 49.962

8.  Infantile cystinosis: a reappraisal of early and late symptoms.

Authors:  M Broyer; M Guillot; M C Gubler; R Habib
Journal:  Adv Nephrol Necker Hosp       Date:  1981

9.  Defective cystine exodus from isolated lysosome-rich fractions of cystinotic leucocytes.

Authors:  W A Gahl; F Tietze; N Bashan; R Steinherz; J D Schulman
Journal:  J Biol Chem       Date:  1982-08-25       Impact factor: 5.157

10.  Binding assays for amino acids. The utilization of a cystine binding protein from Escherichia coli for the determination of acid-soluble cystine in small physiological samples.

Authors:  R G Oshima; R C Willis; C E Furlong; J A Schneider
Journal:  J Biol Chem       Date:  1974-10-10       Impact factor: 5.157
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  3 in total

Review 1.  Update on nephropathic cystinosis.

Authors:  J A Schneider; B Katz; R B Melles
Journal:  Pediatr Nephrol       Date:  1990-11       Impact factor: 3.714

Review 2.  Nephropathic cystinosis: late complications of a multisystemic disease.

Authors:  Galina Nesterova; William Gahl
Journal:  Pediatr Nephrol       Date:  2008-06       Impact factor: 3.714

3.  Long-term tracking of neurological complications of encephalopathy and myopathy in a patient with nephropathic cystinosis: a case report and review of the literature.

Authors:  Marcus Müller; Andrea Baumeier; E B Ringelstein; I W Husstedt
Journal:  J Med Case Rep       Date:  2008-07-18
  3 in total

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