Literature DB >> 11486915

Sustained oral lysine supplementation in ornithine delta-aminotransferase deficiency.

N Elpeleg1, S H Korman.   

Abstract

Oral lysine administration to three patients with B6-nonresponsive gyrate atrophy reduced plasma ornithine concentrations by 21-31% within 1-2 days. No further reduction was noted with time.

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Year:  2001        PMID： 11486915     DOI： 10.1023/a:1010545811361

Source DB:  PubMed          Journal:  J Inherit Metab Dis        ISSN： 0141-8955            Impact factor:   4.982


  2 in total

1.  Oral lysine feeding in gyrate atrophy with hyperornithinaemia--a pilot study.

Authors:  K Peltola; O J Heinonen; K Näntö-Salonen; K Pulkki; O Simell
Journal:  J Inherit Metab Dis       Date:  2000-06       Impact factor: 4.982

2.  Correction of ornithine accumulation prevents retinal degeneration in a mouse model of gyrate atrophy of the choroid and retina.

Authors:  T Wang; G Steel; A H Milam; D Valle
Journal:  Proc Natl Acad Sci U S A       Date:  2000-02-01       Impact factor: 11.205
  2 in total
  3 in total

1.  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

2.  Metabolic follow-up of a Croatian patient with gyrate atrophy and a new mutation in the OAT gene: a case report.

Authors:  Marija Zekušić; Ana Škaričić; Ksenija Fumić; Dunja Rogić; Tamara Žigman; Danijela Petković Ramadža; Nenad Vukojević; Véronique Rüfenacht; Valentina Uroić; Ivo Barić
Journal:  Biochem Med (Zagreb)       Date:  2018-10-15       Impact factor: 2.313

Review 3.  Single amino acid supplementation in aminoacidopathies: a systematic review.

Authors:  Danique van Vliet; Terry G J Derks; Margreet van Rijn; Martijn J de Groot; Anita MacDonald; M Rebecca Heiner-Fokkema; Francjan J van Spronsen
Journal:  Orphanet J Rare Dis       Date:  2014-01-13       Impact factor: 4.123
  3 in total

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