Literature DB >> 8729109

Very long-chain fatty acids in diagnosis, pathogenesis, and therapy of peroxisomal disorders.

H W Moser1, A B Moser.   

Abstract

Abnormally high levels of very long-chain fatty acids (VLCFA) are a feature in nine of the fifteen peroxisomal disorders that have been identified so far. Saturated VLCFA accumulate in X-linked adrenoleukodystrophy, appear to disrupt membrane structure, and may play a role in the pathogenesis of a brain inflammatory response. Dietary therapy initiated when patients are still asymptomatic may be of clinical benefit.

Entities:  

Mesh：

Substances：

Year:  1996        PMID： 8729109     DOI： 10.1007/BF02637066

Source DB:  PubMed          Journal:  Lipids        ISSN： 0024-4201            Impact factor:   1.880


  18 in total

1.  Effect of erucic acid on platelets in patients with adrenoleukodystrophy.

Authors:  T S Kickler; W H Zinkham; A Moser; J Shankroff; J Borel; H Moser
Journal:  Biochem Mol Med       Date:  1996-04

Review 2.  Protein import into peroxisomes and biogenesis of the organelle.

Authors:  S Subramani
Journal:  Annu Rev Cell Biol       Date:  1993

3.  Thrombocytopenia induced by erucic acid therapy in patients with X-linked adrenoleukodystrophy.

Authors:  S Zierz; R Schröder; C J Unkrig
Journal:  Clin Investig       Date:  1993-10

4.  Docosahexaenoic acid--a new therapeutic approach to peroxisomal-disorder patients: experience with two cases.

Authors:  M Martinez; M Pineda; R Vidal; J Conill; B Martin
Journal:  Neurology       Date:  1993-07       Impact factor: 9.910

5.  Membrane microviscosity is increased in the erythrocytes of patients with adrenoleukodystrophy and adrenomyeloneuropathy.

Authors:  R A Knazek; W B Rizzo; J D Schulman; J R Dave
Journal:  J Clin Invest       Date:  1983-07       Impact factor: 14.808

6.  Very long chain fatty acids in X-linked adrenoleukodystrophy brain after treatment with Lorenzo's oil.

Authors:  A Poulos; R Gibson; P Sharp; K Beckman; P Grattan-Smith
Journal:  Ann Neurol       Date:  1994-11       Impact factor: 10.422

7.  A two-year trial of oleic and erucic acids ("Lorenzo's oil") as treatment for adrenomyeloneuropathy.

Authors:  P Aubourg; C Adamsbaum; M C Lavallard-Rousseau; F Rocchiccioli; N Cartier; I Jambaqué; C Jakobezak; A Lemaitre; F Boureau; C Wolf
Journal:  N Engl J Med       Date:  1993-09-09       Impact factor: 91.245

8.  Interactions of a very long chain fatty acid with model membranes and serum albumin. Implications for the pathogenesis of adrenoleukodystrophy.

Authors:  J K Ho; H Moser; Y Kishimoto; J A Hamilton
Journal:  J Clin Invest       Date:  1995-09       Impact factor: 14.808

9.  The inflammatory myelinopathy of adreno-leukodystrophy: cells, effector molecules, and pathogenetic implications.

Authors:  J M Powers; Y Liu; A B Moser; H W Moser
Journal:  J Neuropathol Exp Neurol       Date:  1992-11       Impact factor: 3.685

10.  Dietary erucic acid therapy for X-linked adrenoleukodystrophy.

Authors:  W B Rizzo; R T Leshner; A Odone; A L Dammann; D A Craft; M E Jensen; S S Jennings; S Davis; R Jaitly; J A Sgro
Journal:  Neurology       Date:  1989-11       Impact factor: 9.910
View more
  6 in total

1.  Molecular species of phospholipids with very long chain fatty acids in skin fibroblasts of Zellweger syndrome.

Authors:  Kotaro Hama; Toru Nagai; Chiho Nishizawa; Kazutaka Ikeda; Masashi Morita; Noriko Satoh; Hiroki Nakanishi; Tsuneo Imanaka; Nobuyuki Shimozawa; Ryo Taguchi; Keizo Inoue; Kazuaki Yokoyama
Journal:  Lipids       Date:  2013-12       Impact factor: 1.880

2.  Plasma and red blood cell fatty acids in peroxisomal disorders.

Authors:  A B Moser; D S Jones; G V Raymond; H W Moser
Journal:  Neurochem Res       Date:  1999-02       Impact factor: 3.996

3.  Auditory function in adrenomyeloneuropathy.

Authors:  Joseph P Pillion; Hugo W Moser; Gerald V Raymond
Journal:  J Neurol Sci       Date:  2008-01-15       Impact factor: 3.181

4.  Fast diffusion of very long chain saturated fatty acids across a bilayer membrane and their rapid extraction by cyclodextrins: implications for adrenoleukodystrophy.

Authors:  Biju K Pillai; Ravi Jasuja; Jeffrey R Simard; James A Hamilton
Journal:  J Biol Chem       Date:  2009-09-28       Impact factor: 5.157

5.  Neuronal migration depends on intact peroxisomal function in brain and in extraneuronal tissues.

Authors:  Anneleen Janssen; Pierre Gressens; Markus Grabenbauer; Eveline Baumgart; Arno Schad; Ilse Vanhorebeek; Annelies Brouwers; Peter E Declercq; Dariush Fahimi; Philippe Evrard; Luc Schoonjans; Désiré Collen; Peter Carmeliet; Guy Mannaerts; Paul Van Veldhoven; Myriam Baes
Journal:  J Neurosci       Date:  2003-10-29       Impact factor: 6.167

6.  PEX13 deficiency in mouse brain as a model of Zellweger syndrome: abnormal cerebellum formation, reactive gliosis and oxidative stress.

Authors:  C Catharina Müller; Tam H Nguyen; Barbara Ahlemeyer; Mallika Meshram; Nishreen Santrampurwala; Siyu Cao; Peter Sharp; Pamela B Fietz; Eveline Baumgart-Vogt; Denis I Crane
Journal:  Dis Model Mech       Date:  2010-10-19       Impact factor: 5.758
  6 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.