Zellweger syndrome knockout mouse models challenge putative peroxisomal β-oxidation involvement in docosahexaenoic acid (22:6n-3) biosynthesis

Molecular Genetics and Metabolism

Volumn 72, Issue 1, 2001, Pages 1-7

  Infante, Juan P ^a   Huszagh, Virginia A ^a  

^a Institute for Theoretical Biochemistry and Molecular Biology   (United States)

Author keywords

Carnitine; Craniofacial dysmorphisms; Desaturase; Hepatomegaly; Hypotonia; Mental retardation; Mitochondria; Myelin; Neuronal migration; Peroxisomes

Indexed keywords

ACYL COENZYME A OXIDASE; CARNITINE; DIHYDROXYACETONE PHOSPHATE ACYLTRANSFERASE; DOCOSAHEXAENOIC ACID; MYELIN; PEROXIN; PLASMALOGEN;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; BRAIN DEVELOPMENT; BRAIN MITOCHONDRION; CELL MIGRATION; CONTROLLED STUDY; CRANIOFACIAL MALFORMATION; DIET SUPPLEMENTATION; DISORDERS OF PEROXISOMAL FUNCTIONS; ENZYME ACTIVITY; FATTY ACID OXIDATION; FATTY ACID SYNTHESIS; GENE DISRUPTION; GENE MUTATION; HEPATOMEGALY; KNOCKOUT MOUSE; LIPID ANALYSIS; LIVER CELL CULTURE; MENTAL RETARDATION MALFORMATION SYNDROME; MOLECULAR RECOGNITION; MOUSE; MUSCLE HYPOTONIA; MYELINATION; NERVE CELL; NONHUMAN; PATHOPHYSIOLOGY; PEROXISOME; PHENOTYPE; PRIORITY JOURNAL; SHORT SURVEY; ZELLWEGER SYNDROME;

EID: 0035718734     PISSN: 10967192     EISSN: None     Source Type: Journal    
DOI: 10.1006/mgme.2000.3101     Document Type: Article

References (50)

1. Polyunsaturated fatty acids in the developing brain, erythrocytes and plasma in peroxisomal disease: Therapeutic implications
2. Developmental changes in rat brain membrane lipids and fatty acids: The preferential prenatal accumulation of docosahexaenoic acid
3. Fatty acid composition of late embryonic and early postnatal rat brain
4. Abnormal profiles of polyunsaturated fatty acids in the brain, liver, kidney and retina of patients with peroxisomal disorders
5. Peroxisomal and mitochondrial defects in the cerebro-hepato-renal syndrome
6. The cerebrohepatorenal (Zellweger) syndrome
7. A familial syndrome of multiple congenital defects
8. Peroxisomal disorders: Overview
9. Peroxisomal disorders: Genotype, phenotype, major neuropathologic lesions, and pathogenesis
10. Genotype-phenotype correlations in disorders of peroxisome biogenesis
11. Phospholipid supplementation reverses behavioral and biochemical alterations induced by n- 3 polyunsaturated fatty acid deficiency in mice
12. Fatty acid composition of brain phospholipids in aging and in Alzheimer's disease
13. 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
14. 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
15. 4-desaturase activity in cultured skin fibroblasts
16. 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
17. Targeted deletion of the PEX2 peroxisome assembly gene in mice provides a model for Zellweger syndrome, a human neuronal migration disorder
18. A mouse model for Zellweger syndrome
19. Glycerolipid biosynthesis in peroxisomes (micro-bodies)
20. A function for the vitamin E metabolite α-to-copherol quinone as an essential enzyme cofactor for the mitochondrial fatty acid desaturases
21. Inhibitory effects of longchain unsaturated fatty acids on mitochondrial β-oxidation. Effects of streptozotocin-induced diabetes on mitochondrial β-oxidation of polyunsaturated fatty acids
22. Membrane docosahexaenoic is supplied to the developing brain and retina by the liver
23. Low docosahexaenoic acid in brain but not in plasma and red blood cell in newborn Zellweger syndrome mouse
24. Docosahexaenoic acid deficit is not a major pathogenic factor in peroxisome-deficient mice
25. 38 polyenoic fatty acids in the brain of patients without peroxisomes (Zellweger's syndrome)
26. 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
27. 14]acetate in normal and peroxisome-deficient (Zellweger) fibroblasts
28. Effects of dietary n- 3 fatty acids on the phospholipid molecular species of monkey brain
29. Severe plasmalogen deficiency in tissues of infants without peroxisomes (Zellweger syndrome)
30. Plasmalogen status influences docosahexaenoic acid levels in a macrophage cell line: Insights using ether lipid-deficient variants
31. Generalized peroxisomal disorder in male twins: Fatty acid composition of serum lipids and response to n- 3 fatty acids
32. Therapeutic effects of docosahexaenoic acid ethyl ester in patients with generalized peroxisomal disorders
33. MRI evidence that docosahexaenoic acid ethyl ester improves myelination in generalized peroxisomal disorders
34. The acyl and alk-1-enyl groups of the major phosphoglycerides from ox brain myelin and mouse brain microsomal, mitochondrial and myelin fractions
35. Fatty acid and fatty aldehyde composition of the major brain lipids in normal human gray matter, white matter, and myelin
36. Lipids of nervous tissue: Composition and metabolism
37. Postnatal development and isolation of peroxisomes from brain
38. 14 isotope from acetate and palmitate into the α,β-unsaturated ether chain
39. Physiological significance of fatty acid elongation system in adrenoleukodystrophy
40. 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
41. L-carnitine
42. Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency
43. Longchain L-3-hydroxyacyl-coenzyme A dehydrogenase deficiency neuropathy: Response to cod liver oil
44. Dietary management of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD). A case report and survey
45. Docosahexaenoic acid and retinal function in children with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency
46. Hepatocellular and hepatic peroxisomal alterations in mice with a disrupted peroxisomal fatty acyl-coenzyme A oxidase gene
47. Myelination in rat brain: Changes in myelin composition during brain maturation
48. Δ6 desaturase in brain and liver during development and aging
49. Elongation, desaturation, and esterification of essential fatty acids by fetal rat brain in vivo
50. Phenotypes of patients with "simple" Mendelian disorders are complex traits: Thresholds, modifiers, and systems dynamics