Mitochondrial trifunctional protein defects: Clinical implications and therapeutic approaches
- 4 July 2008
- journal article
- review article
- Published by Elsevier BV in Advanced Drug Delivery Reviews
- Vol. 60 (13-14), 1488-1496
- https://doi.org/10.1016/j.addr.2008.04.014
Abstract
No abstract availableKeywords
This publication has 88 references indexed in Scilit:
- Mitochondrial protein import and human health and diseaseBiochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, 2006
- Metabolic control during exercise with and without medium-chain triglycerides (MCT) in children with long-chain 3-hydroxy acyl-CoA dehydrogenase (LCHAD) or trifunctional protein (TFP) deficiencyMolecular Genetics and Metabolism, 2006
- TAT-mediated intracellular delivery of purine nucleoside phosphorylase corrects its deficiency in miceJCI Insight, 2006
- Uric acid and anti-TNF antibody improve mitochondrial dysfunction in ob/ob miceHepatology, 2006
- Defective hepatic mitochondrial respiratory chain in patients with nonalcoholic steatohepatitisHepatology, 2003
- Role of AMP-activated protein kinase in mechanism of metformin actionJCI Insight, 2001
- Retroviral Vectors for Liver-directed Gene TherapySeminars in Liver Disease, 1999
- Intact α-Subunit Is Required for Membrane Binding of Human Mitochondrial Trifunctional β-Oxidation Protein, but Is Not Necessary for Conferring 3-Ketoacyl-CoA Thiolase Activity to the β-SubunitBiochemical and Biophysical Research Communications, 1995
- Molecular basis of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: identification of the major disease-causing mutation in the α-subunit of the mitochondrial trifunctional proteinBiochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism, 1994
- Human liver long-chain 3-hydroxyacyl-coenzyme a dehydrogenase is a multifunctional membrane-bound beta-oxidation enzyme of mitochondriaBiochemical and Biophysical Research Communications, 1992