Zellweger Syndrome Knockout Mouse Models Challenge Putative Peroxisomal β-Oxidation Involvement in Docosahexaenoic Acid (22:6n-3) Biosynthesis

Peroxisomal and Mitochondrial Defects in the Cerebro-Hepato-Renal Syndrome

1973 • 777 citations

Lipids of nervous tissue: Composition and metabolism

1985 • 725 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

MYELINATION IN RAT BRAIN: CHANGES IN MYELIN COMPOSITION DURING BRAIN MATURATION¹

1973 • 655 citations

Fatty acid composition of brain phospholipids in aging and in Alzheimer’s disease

1991 • 645 citations

Fatty acid and fatty aldehyde composition of the major brain lipids in normal human gray matter, white matter, and myelin

1965 • 471 citations

Membrane docosahexaenoate is supplied to the developing brain and retina by the liver.

1989 • 437 citations

Phenotypes of Patients with “Simple” Mendelian Disorders Are Complex Traits: Thresholds, Modifiers, and Systems Dynamics

2000 • 418 citations

Severe plasmalogen deficiency in tissues of infants without peroxisomes (Zellweger syndrome)

1983 • 340 citations

A mouse model for Zellweger syndrome

1997 • 271 citations

The Cerebrohepatorenal (Zellweger) Syndrome

1984 • 268 citations

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

1996 • 246 citations

A FAMILIAL SYNDROME OF MULTIPLE CONGENITAL DEFECTS.

1964 • 235 citations

Peroxisomal Disorders: Genotype, Phenotype, Major Neuropathologic Lesions, and Pathogenesis

1998 • 200 citations

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

1992 • 187 citations

Phospholipid supplementation reverses behavioral and biochemical alterations induced by n–3 polyunsaturated fatty acid deficiency in mice

2000 • 158 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

Developmental changes in rat brain membrane lipids and fatty acids: the preferential prenatal accumulation of docosahexaenoic acid

1999 • 144 citations

Δ6 desaturase in brain and liver during development and aging

1990 • 121 citations

MRI evidence that docosahexaenoic acid ethyl ester improves myelination in generalized peroxisomal disorders

1998 • 120 citations

Therapeutic effects of docosahexaenoic acid ethyl ester in patients with generalized peroxisomal disorders

2000 • 118 citations

Genotype–Phenotype Correlations in Disorders of Peroxisome Biogenesis

1999 • 98 citations

The acyl and Alk‐1‐enyl groups of the major phosphoglycerides from ox brain myelin and mouse brain microsomal, mitochondrial and myelin fractions

1970 • 93 citations

Secondary carnitine deficiency and impaired docosahexaenoic (22:6n‐3) acid synthesis: a common denominator in the pathophysiology of diseases of oxidative phosphorylation and β‐oxidation

2000 • 92 citations

Long‐chain 3‐hydroxyacyl‐CoA dehydrogenase deficiency

1999 • 90 citations

Dietary management of long‐chain 3‐hydroxyacyl‐CoA dehydrogenase deficiency (LCHADD). A case report and survey

1999 • 83 citations

Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency

1999 • 79 citations

Effects of Dietary n‐3 Fatty Acids on the Phospholipid Molecular Species of Monkey Brain

1990 • 71 citations

Fatty acid composition of late embryonic and early postnatal rat brain

1996 • 70 citations

Glycerolipid biosynthesis in peroxisomes (microbodies)

1995 • 69 citations

Detection of a homologous series of C26-C38 polyenoic fatty acids in the brain of patients without peroxisomes (Zellweger's syndrome)

1986 • 64 citations

Docosahexaenoic acid and retinal function in children with long‐chain 3‐hydroxyacyl‐CoA dehydrogenase deficiency

1999 • 60 citations

Analysis of the putative role of 24‐carbon polyunsaturated fatty acids in the biosynthesis of docosapentaenoic (22:5n‐6) and docosahexaenoic (22:6n‐3) acids

1998 • 60 citations

Postnatal Development and Isolation of Peroxisomes from Brain

1991 • 60 citations

Plasmalogens of the nervous system. I. Deposition in developing rat brain and incorporation of C14 isotope from acetate and palmitate into the α,β-unsaturated ether chain

1959 • 60 citations

On the molecular etiology of decreased arachidonic (20:4n-6), docosapentaenoic (22:5n-6) and docosahexaenoic (22:6n-3) acids in Zellweger syndrome and other peroxisomal disorders.

1997 • 59 citations

Peroxisomal β-oxidation of Polyunsaturated Long Chain Fatty Acids in Human Fibroblasts. The Polyunsaturated and the Saturated Long Chain Fatty Acids are Retroconverted by the Same Acyl-CoA Oxidase

1993 • 59 citations

Elongation, desaturation, and esterification of essential fatty acids by fetal rat brain in vivo.

1993 • 58 citations

Inhibitory effects of some long-chain unsaturated fatty acids on mitochondrial β-oxidation. Effects of streptozotocin-induced diabetes on mitochondrial β-oxidation of polyunsaturated fatty acids

1985 • 57 citations

L-Carnitine.

1996 • 56 citations

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

1995 • 55 citations

Plasmalogen status influences docosahexaenoic acid levels in a macrophage cell line: insights using ether lipid-deficient variants

1999 • 54 citations

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

2000 • 53 citations

A function for the vitamin E metabolite α‐tocopherol quinone as an essential enzyme cofactor for the mitochondrial fatty acid desaturases

1999 • 51 citations

Peroxisomal Disorders: Overview

1996 • 40 citations

The zellweger syndrome: Deficient conversion of docosahexaenoic acid (22:6(n−3)) to eicosapentaenoic acid (20:5(n−3)) and normal Δ4-desaturase activity in cultured skin fibroblasts

1990 • 37 citations

Physiological significance of fatty acid elongation system in adrenoleukodystrophy

1991 • 34 citations

Long-chain <scp>l</scp> -3-hydroxyacyl-coenzyme A dehydrogenase deficiency neuropathy: Response to cod liver oil

1999 • 26 citations

Changes in blood and plasma volume during treatment with recombinant human erythropoietin

1993 • 18 citations

Generalized peroxisomal disorder in male twins: Fatty acid composition of serum lipids and response to n−3 fatty acids

1998 • 12 citations

Fatty acid synthesis from [2‐¹⁴C]acetate in normal and peroxisome‐deficient (Zellweger) fibroblasts

1993 • 7 citations