Literature DB >> 4058549

Long-term treatment of infantile nephropathic cystinosis with cysteamine.

V A da Silva, R P Zurbrügg, P Lavanchy, A Blumberg, H Suter, S R Wyss, C M Lüthy, O H Oetliker.   

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Year:  1985        PMID： 4058549     DOI： 10.1056/NEJM198512053132307

Source DB:  PubMed          Journal:  N Engl J Med        ISSN： 0028-4793            Impact factor:   91.245


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  13 in total

1.  Infantile nephropathic cystinosis presenting as incomplete Fanconi syndrome and refractory rickets.

Authors:  V S Rajadurai; P Shanbag; M S Seshadri; U Khanduri; T A Alexander; M A Jadhav
Journal:  Indian J Pediatr       Date:  1989 May-Jun       Impact factor: 1.967

2.  Sialic acid storage diseases. A multiple lysosomal transport defect for acidic monosaccharides.

Authors:  G M Mancini; C E Beerens; P P Aula; F W Verheijen
Journal:  J Clin Invest       Date:  1991-04       Impact factor: 14.808

Review 3.  Update on nephropathic cystinosis.

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

4.  Cystinosin, the small GTPase Rab11, and the Rab7 effector RILP regulate intracellular trafficking of the chaperone-mediated autophagy receptor LAMP2A.

Authors:  Jinzhong Zhang; Jennifer L Johnson; Jing He; Gennaro Napolitano; Mahalakshmi Ramadass; Celine Rocca; William B Kiosses; Cecilia Bucci; Qisheng Xin; Evripidis Gavathiotis; Ana María Cuervo; Stephanie Cherqui; Sergio D Catz
Journal:  J Biol Chem       Date:  2017-05-02       Impact factor: 5.157

5.  Upregulation of the Rab27a-dependent trafficking and secretory mechanisms improves lysosomal transport, alleviates endoplasmic reticulum stress, and reduces lysosome overload in cystinosis.

Authors:  Jennifer L Johnson; Gennaro Napolitano; Jlenia Monfregola; Celine J Rocca; Stephanie Cherqui; Sergio D Catz
Journal:  Mol Cell Biol       Date:  2013-05-28       Impact factor: 4.272

Review 6.  Nephropathic cystinosis: late complications of a multisystemic disease.

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

7.  Progression of chronic renal failure in a historical group of patients with nephropathic cystinosis. European Collaborative Study on Cystinosis.

Authors:  F Manz; N Gretz
Journal:  Pediatr Nephrol       Date:  1994-08       Impact factor: 3.714

8.  Complications of nephropathic cystinosis after renal failure.

Authors:  W A Gahl; M I Kaiser-Kupfer
Journal:  Pediatr Nephrol       Date:  1987-07       Impact factor: 3.714

9.  Impairment of chaperone-mediated autophagy leads to selective lysosomal degradation defects in the lysosomal storage disease cystinosis.

Authors:  Gennaro Napolitano; Jennifer L Johnson; Jing He; Celine J Rocca; Jlenia Monfregola; Kersi Pestonjamasp; Stephanie Cherqui; Sergio D Catz
Journal:  EMBO Mol Med       Date:  2015-02       Impact factor: 12.137

10.  Chaperone-Mediated Autophagy Upregulation Rescues Megalin Expression and Localization in Cystinotic Proximal Tubule Cells.

Authors:  Jinzhong Zhang; Jing He; Jennifer L Johnson; Farhana Rahman; Evripidis Gavathiotis; Ana Maria Cuervo; Sergio D Catz
Journal:  Front Endocrinol (Lausanne)       Date:  2019-02-01       Impact factor: 5.555
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