Mitochondrial alterations caused by defective peroxisomal biogenesis in a mouse model for Zellweger syndrome (PEX5 knockout mouse)

American Journal of Pathology

Volumn 159, Issue 4, 2001, Pages 1477-1494

  Baumgart, Eveline ^a   Vanhorebeek, Ilse ^b   Grabenbauer, Markus ^a   Borgers, Marcel ^a,c,d   Declercq, Peter E ^b   Fahimi, H Dariush ^a   Baes, Myriam ^b  

^a UNIVERSITY OF HEIDELBERG   (Germany)

^b UNIVERSITY OF LEUVEN   (Belgium)

^c JANSSEN RESEARCH FOUNDATION   (Belgium)

^d MAASTRICHT UNIVERSITY   (Netherlands)

Author keywords

[No Author keywords available]

Indexed keywords

MANGANESE SUPEROXIDE DISMUTASE; MATRIX PROTEIN; REACTIVE OXYGEN METABOLITE;

ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; BIOGENESIS; CELL ULTRASTRUCTURE; COMPLEX FORMATION; CONTROLLED STUDY; FATTY ACID OXIDATION; HYPOXIA; IMMUNOCYTOCHEMISTRY; LIPID METABOLISM; MITOCHONDRIAL MEMBRANE; MOUSE; NONHUMAN; PATHOGENESIS; PEROXISOME; PRIORITY JOURNAL; REOXYGENATION; RESPIRATORY CHAIN; ZELLWEGER SYNDROME;

EID: 0034799180     PISSN: 00029440     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0002-9440(10)62534-5     Document Type: Article

References (90)

1. A mouse model for Zellweger syndrome
2. Peroxisomes (microbodies and related particles)
3. Mammalian peroxisomes: Metabolism of oxygen and reactive oxygen species
4. Stress induces peroxisome biogenesis genes
5. Induction of tubular peroxisomes by UV irradiation and reactive oxygen species in HepG2 cells
6. Functions and organization of peroxisemal β-oxidation
7. Role of fatty acids in signal transduction: Modulators and messengers
8. Cellular signalling: The role of the peroxisome
9. Omega-3 polyunsaturated fatty acid regulation of gene expression
10. Cell compartmentalization of cholesterol biosynthesis
11. Lipid biosynthesis in peroxisomes
12. Peroxisomal and mitochondrial defects in the cerebro-hepato-renal syndrome
13. Peroxisomal defects in neonatal-onset and X-linked adrenoleukodystrophies
14. Molecular genetics of perexisomal disorders
15. Disorders of peroxisome biogenesis
16. The human peroxisomal targeting receptor, Pex 5p, is translocated into the peroxisomal matrix and recycled to the cytosol
17. Peroxisome biogenesis disorders
18. Neuronal migration disorder in Zellweger mice is secondary to glutamate receptor dysfunction
19. Docosahexaenoic acid deficit is not a major pathogenic factor in peroxisome-deficient mice
20. Ultrastructure of the liver in the cerebrohepatorenal syndrome of Zellweger
21. The cerebrohepatorenal syndrome of Zellweger, morphologic and metabolic aspects
22. Pathology of hepatic peroxisomes and mitochondria in patients with peroxisomal disorders
23. Biochemical studies in the liver and muscle of patients with Zellweger syndrome
24. Multiple peroxisomal enzymatic deficiency disorders. A comparative biochemical morphologic study of Zellweger cerebrohepatorenal syndrome and neonatal adrenoleukodystrophy
25. Selective induction of peroxisomal enzymes by the hypolipidemic drug bezafibrate. Detection of modulations by automatic image analysis in conjunction with immunoelectron microscopy and immunoblotting
26. Immunohistochemical localization of superoxide dismutases in fetal and neonatal rat tissues
27. Detection of peroxisomal proteins and their mRNAs in fetal and newborn mice
28. Amplification methods to increase the sensitivity of in situ hybridization: Play card(s)
29. Nonradioactive in situ hybridization for detection of mRNAs encoding for peroxisomal proteins: Heterogeneous hepatic lobular distribution after treatment with a single dose of bezafibrate
30. Use of ferrocyanide reduced osmium tetroxide in electron microscopy
31. Cytochemical localization of catalase in rat hepatic microbodies (peroxisomes)
32. Selective cytochemical localization of peroxidase, cytochrome oxidase and catalase in rat with 3,3′-diaminobenzidine
33. Nondroplet ultrastructural demonstration of cytochrome oxidase activity with a polymerizing osmiophilic reagent, diaminobenzidine (DAB)
34. Ultrastructural localization of several phosphatases with cerium
35. Biogenesis of peroxisomes: Immunocytochemical investigation of peroxisomal membrane proteins in proliferating rat liver peroxisomes and in catalase-negative membrane loops
36. A simple post-embedding system for the rapid demonstration of tissue antigens under the electron microscope
37. Contributions of the immunogold technique to investigation of the biology of peroxisomes
38. Comparison of LR White and Unicryl as embedding media for light and electron immunomicroscopy of chromaffin cells
39. Morphology of peroxisomes in light- and electron microscopy
40. Sizing of protein A-colloidal gold probes for immunoelectron microscopy
41. Leigh syndrome: Clinical features and biochemical and DNA abnormalities
42. Adenosine 5′-triphosphate. UV-method with phosphoglycerate kinase
43. L-(+)-lactate
44. Pyruvate
45. Evolution of pyruvate carboxylase and other biotin containing enzymes in developing rat liver and kidney
46. Mitochondrial complex I deficiency leads to increased production of superoxide radicals and induction of superoxide dismutase
47. A metabolic disorder similar to Zellweger syndrome with hepatic acatalasia and absence of peroxisomes, altered content and redox state of cytochromes, and infantile cirrhosis with hemosiderosis
48. Zellweger syndrome: Biochemical and morphological studies on two patients treated with clofibrate
49. The cerebro-hepato-renal (Zellweger) syndrome: Lamellar lipid profiles in adrenocortical, hepatic mesenchymal, astrocyte cells and increased levels of very long chain fatty acids and phytanic acid in the plasma
50. Mitochondrial myopathy of cerebro-hepato-renal (Zellweger) syndrome
51. Mitochondrial myopathy with loosely coupled oxidative phosphorylation in a case of Zellweger syndrome
52. Pathology of peroxisomal disorders
53. Peroxisomal disorders: Genotype, phenotype, major neuropathological lesions, and pathogenesis
54. The pathology of peroxisomal disorders with pathogenetic considerations
55. The regulation of mitochondrial oxygen uptake by redox reactions involving nitric oxide and ubiquinol
56. NADH-coenzyme Q reductase (complex I) deficiency: Heterogeneity in phenotype and biochemical findings
57. Disorders of the mitochondria
58. Threshold effect and tissue specificity Implications for mitochondrial cytophathies
59. Hydroperoxide metabolism in mammalian organs
60. Hepatotoxicity due to mitochondrial dysfunction
61. Mitochondriopathy
62. Mitochondria. Ultrastructural pathology of the cell
63. The Liver
64. Mitochondria in the etiology and pathogenesis of Parkinson's disease
65. The effect of iron overload on rat plasma and liver oxidant status in vivo
66. Fetal cerebrohepatorenal (Zellweger syndrome): Dysmorphic, radiologic, biochemical, and pathologic findings in four affected fetuses
67. Onset of maternal arterial blood flow and placental oxidative stress. A possible factor in human early pregnancy failure
68. The role of manganese superoxide dismutase in health and disease, review
69. The use of transgenic mice to study oxygen free radical metabolism
70. Titrating the effects of mitochondrial complex I impairment in the cell physiology
71. The oxidative inactivation of mitochondrial electron transport chain components and ATPase
72. Targeted deletion of the PEX2 peroxisome assembly gene in mice provides a model for Zellweger syndrome, a human neuronal migration disorder
73. Peroxisomes as a source of reactive oxygen species and nitric oxide signal molecules in plant cells
74. Localization of xanthine oxidase in crystalline cores of peroxisomes. A cytochemical and biochemical study
75. Cu, Zn superoxide dismutase is a peroxisomal enzyme in human fibroblasts and hepatoma cells
76. Manganese superoxide dismutase in rat liver peroxisomes: Biochemical and immunochemical evidence
77. Demonstration of glutathione peroxidase in rat liver peroxisomes and its intraorganellar distribution
78. Carcinogenesis by hepatic peroxisome proliferators: Evaluation of the risk of hypolipidemic drugs and industrial plasticizers to humans
79. Hepatocellular and hepatic peroxisomal alterations in mice with a disrupted peroxisomal fatty acyl-coenzyme A oxidase gene
80. Peroxisomal and mitochondrial fatty acid beta-oxidation in mice nullizygous for both peroxisome proliferator-activated receptor alpha and peroxisomal fatty acyl-CoA oxidase. Genotype correlation with fatty liver phenotype
81. Ischemia-reperfusion injury: Biochemical alterations in peroxisomes of rat kidney
82. Mitochondrial dysfunction during hypoxia/reoxygenation and its correction by anaerobic metabolism of citric acid cycle intermediates
83. Photosensitized killing of cultured fibroblasts from patients with peroxisomal disorders due to pyrene fatty acid-mediated ultraviolet damage
84. Evidence that photodynamic stress kills Zellweger fibroblasts by a nonapoptotic mechanism
85. Ether lipids in biomembranes
86. Heterogeneity of peroxisomes in human hepatoblastoma cell line HepG2. Evidence of distinct subpopulations
87. Reduced antioxidative capacity in liver mitochondria from bile duct ligated rats
88. Interactions of a very long chain fatty acid with model membranes and serum albumin. Implications for the pathogenesis of adrenoleukodystrophy
89. Liver in the cerebro-hepato-renal syndrome: Defective bile acid synthesis and abnormal mitochondria