The Neuronal Migration Defect in Mice with Zellweger Syndrome (Pex5Knockout) is not Caused by the Inactivity of Peroxisomal β-Oxidation

Rapid separation of lipid classes in high yield and purity using bonded phase columns.

1985 • 939 citations

The metabolism of 7,10,13,16,19-docosapentaenoic acid to 4,7,10,13,16,19-docosahexaenoic acid in rat liver is independent of a 4-desaturase.

1991 • 660 citations

Steatohepatitis, Spontaneous Peroxisome Proliferation and Liver Tumors in Mice Lacking Peroxisomal Fatty Acyl-CoA Oxidase

1998 • 341 citations

Plasma very long chain fatty acids in 3,000 peroxisome disease patients and 29,000 controls

1999 • 334 citations

A mouse model for X-linked adrenoleukodystrophy

1997 • 277 citations

A mouse model for Zellweger syndrome

1997 • 271 citations

Hepatocellular and Hepatic Peroxisomal Alterations in Mice with a Disrupted Peroxisomal Fatty Acyl-coenzyme A Oxidase Gene

1996 • 246 citations

Targeted inactivation of the X-linked adrenoleukodystrophy gene in mice

1997 • 206 citations

Inactivation of the Peroxisomal Multifunctional Protein-2 in Mice Impedes the Degradation of Not Only 2-Methyl-branched Fatty Acids and Bile Acid Intermediates but Also of Very Long Chain Fatty Acids

2000 • 192 citations

Abnormal profiles of polyunsaturated fatty acids in the brain, liver, kidney and retina of patients with peroxisomal disorders

1992 • 187 citations

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

1995 • 184 citations

Adrenoleukodystrophy Protein-Deficient Mice Represent Abnormality of Very Long Chain Fatty Acid Metabolism

1997 • 184 citations

Effect of harvesting methods, growth conditions and growth phase on diacylglycerol levels in cultured human adherent cells

1988 • 153 citations

Targeted Deletion of the PEX2 Peroxisome Assembly Gene in Mice Provides a Model for Zellweger Syndrome, a Human Neuronal Migration Disorder

1997 • 152 citations

The peroxisome biogenesis disorders

2001 • 130 citations

Peroxisomal Lipid Degradation via β and β-oxidation in Mammals

2000 • 123 citations

Rapid stable isotope dilution analysis of very-long-chain fatty acids, pristanic acid and phytanic acid using gas chromatography–electron impact mass spectrometry

1998 • 99 citations

Peroxisomal Straight-chain Acyl-CoA Oxidase and D-bifunctional Protein Are Essential for the Retroconversion Step in Docosahexaenoic Acid Synthesis

2001 • 98 citations

Absence of Spontaneous Peroxisome Proliferation in Enoyl-CoA Hydratase/l-3-Hydroxyacyl-CoA Dehydrogenase-deficient Mouse Liver

1999 • 96 citations

The pathology of peroxisomal disorders with pathogenetic considerations.

1995 • 71 citations

PRESIDENTIAL ADDRESS

1995 • 65 citations

Mitochondrial and peroxisomal beta oxidation of the branched chain fatty acid 2-methylpalmitate in rat liver.

1991 • 60 citations

Neuronal migration disorder in Zellweger mice is secondary to glutamate receptor dysfunction

2000 • 59 citations

The Peroxisome Deficient PEX2 Zellweger Mouse: Pathologic and Biochemical Correlates of Lipid Dysfunction

2001 • 58 citations

A new neonatal case ofN‐acetylglutamate synthase deficiency treated by carbamylglutamate

1994 • 57 citations

Polyunsaturated fatty acids in the developing human brain, erythrocytes and plasma in peroxisomal disease: therapeutic implications

1995 • 55 citations

Neuronal migration abnormality in peroxisomal bifunctional enzyme defect

1996 • 55 citations

Docosahexaenoic Acid Deficit Is Not a Major Pathogenic Factor in Peroxisome-Deficient Mice

2000 • 53 citations

Structural and Chemical Alterations in the Cerebral Maldevelopment of Fetal Cerebro-Hepato-Renal (Zellweger) Syndrome

1989 • 49 citations

Prenatal and postnatal development of peroxisomal lipid-metabolizing pathways in the mouse

2001 • 47 citations

The deficient degradation of synthetic 2‐ and 3‐methyl‐branched fatty acids in fibroblasts from patients with peroxisomal disorders

1993 • 43 citations

Prenatal and postnatal development of peroxisomal lipid-metabolizing pathways in the mouse

2001 • 24 citations