Mouse Models for Peroxisome Biogenesis Disorders

Cell Biochemistry and Biophysics

Volumn 32, Issue , 2000, Pages 229-237

  Baes, Myriam ^a  

^a UNIVERSITY OF LEUVEN   (Belgium)

Author keywords

Knockout mice; Neuronal migration; Peroxisome; Zellweger

Indexed keywords

ANIMAL; DISEASE MODEL; DISORDERS OF PEROXISOMAL FUNCTIONS; HUMAN; MOUSE; REVIEW;

ANIMALS; DISEASE MODELS, ANIMAL; HUMANS; MICE; PEROXISOMAL DISORDERS;

EID: 0034576334     PISSN: 10859195     EISSN: None     Source Type: Journal    
DOI: 10.1385/CBB:32:1-3:229     Document Type: Article

References (26)

1. Subramani, S. (1998) Components involved in peroxisome import, biogenesis, proliferation, turnover, and movement. Physiol. Rev. 78, 171-188.
2. Powers, J. M. and Moser, H. W. (1998) Peroxisomal disorders: genotype, phenotype, major neuropathologic lesions, and pathogenesis. Brain Pathol. 8, 101-120.
3. Lazarow, P. B. and Moser, H. W. (1989) Disorders of peroxisome biogenesis, in The Metabolic Basis of Inherited Disease (Beaudet, A. L., Scriver, C. R., Sly, W. S., and Valle, D., eds.) McGraw Hill, New York, pp. 1479-1509.
4. Evrard, P., Caviness, V. S., Prats-Vinas, J., and Lyon, G. (1978) The mechanism of arrest of neuronal migration in the Zellweger malformation: an hypothesis based upon cytoarchitectonic analysis. Acta Neuropathol. 41, 109-117.
5. Moser, A. B., Rasmussen, M., Naidu, S., Watkins, P. A., McGuinness, M., Hajra, A. K., et al. (1995) Phenotype of patients with peroxisomal disorders subdivided into sixteen complementation groups. J. Pediatr. 127, 13-22.
6. Mannaerts, G. P. and Van Veldhoven, P. P. (1993) Metabolic role of mammalian peroxisomes, in Peroxisomes: Biology and importance in toxicology and medicine (Gibson, G. and Lake, B., eds), Taylor and Francis, London, pp. 19-62.
7. Reddy, J. K. and Mannaerts, G. P. (1994) Peroxisomal lipid metabolism. Annu. Rev. Nutr. 14, 343-370.
8. Faust, P. L. and Hatten, M. E. (1997) Targeted deletion of PEX2 peroxisome assembly gene in mice provides a model for Zellweger syndrome, a human neuronal migration disorder. J. Cell Biol. 139, 1293-1305.
9. Baes, M., Gressens, P., Baumgart, E., Carmeliet, P., Casteels, M., Fransen, M., et al. (1997) A mouse model for Zellweger syndrome. Nature Genet. 17, 49-56.
10. Tsukamoto, T, Miura, S., and Fujiki, Y. (1991) Restoration by a 35K membrane protein of peroxisome assembly in a peroxisome-deficient mammalian cell mutant. Nature 350, 77-81.
11. Slawecki, M. L., Dodt, G., Steinberg, S., Moser, A. B., Moser, H. W., and Gould, S. J. (1995) Identification of three distinct peroxisomal protein import defects in patients with peroxisome biogenesis disorders. J. Cell Sci. 108, 1817-1829.
12. Dodt, G., Braverman, N., Wong, C., Moser, A., Moser, H. W., Watkins, P., Valle, D., and Gould, S. J. (1995) Mutations in the PTS1 receptor gene,PXR1, define complementation group 2 of the peroxisome biogenesis disorders. Nature Genet. 9, 115-125.
13. Wiemer, E. A. C., Nuttley, W. M., Bertolact, B. L., Li, X., Francke, U., Wheelock, M. J., et al. (1995) Human peroxisomal targeting signal-1 receptor restores peroxisomal protein import in cells from patients with fatal peroxisomal disorders. J. Cell Biol. 130, 51-65.
14. Otera, H., Okumoto, K., Tateishi, K., Ikoma, Y., Matsuda, E., Nishimura, M., et al. (1998) Peroxisome targeting signal type 1 (PTS1) receptor is involved in import of both PTS1 and PTS2: studies with PEX5-defective CHO cell mutants. Mol. Cell Biol. 18, 388-399.
15. Brocard, C., Kragler, F., Simon, M. M., Schuster, T., and Hartig, A. (1994) The tetratricopeptide repeat-domain of the PAS10 protein of Saccharomyces cerevisiae is essential for binding the peroxisomal targeting signal-SKL. Biochem. Biophys. Res. Commun. 204, 1016-1022.
16. Nagy, A., Rossant, J., Nagy, R., Abramow-Newerly, W., and Roder, J. C. (1993) Derivation of completely cell culture-derived mice from early-passage embryonic stem cells. Proc. Natl. Acad. Sci. USA 90, 8424-8428.
17. Johnson, A. B., Schaumburg, H. H., and Powers, J. M. (1976) Histochemical characteristics of the striated inclusions of adrenoleukodystrophy. J. Histochemistry Cytochemistry 24, 725-730.
18. Powers, J. M. (1995) The pathology of peroxisomal disorders with pathogenetic considerations. J. Neuropathol. Exp. Neurol. 54, 710-719.
19. Fahimi, H. D. (1969) Cytochemical localization of peroxidatic activity of catalase in rat hepatic microbodies (peroxisomes). J. Cell Biol. 43, 275-288.
20. Santos, M. J., Imanaka, T., Shio, H., Small, G. M., and Lazarow, P. B. (1988) Peroxisomal membrane ghosts in Zellweger syndrome: aberrant organelle assembly. Science 239, 1536.
21. Wiemer, E. A. C., Brul, S., Just, W. W., Van Driel, R., Brouwer-Kelder, E., Van Den Berg, M., et al. (1989) Presence of peroxisomal membrane proteins in liver and fibroblasts from patients with the Zellweger syndrome and related disorders: Evidence for the existence of peroxisomal ghosts. Eur. J. Cell Biol. 50, 407-417.
22. Goldfisher, S., Moore, C. L., Johnson, A. B., Spiro, A. J., Valsamis, M. P., Wisniewskin, H. K., et al. (1973) Peroxisomal and mitochondrial defects in the cerebro-hepato-renal syndrome. Science 182, 62-64.
23. Powers, J. M., Tummons, R. C., Caviness, V. S., Moser, A. B., and Moser, H. W. (1989) Structural and chemical alterations in the cerebral maldevelopment of fetal cerebro-hepato-renal (Zellweger) syndrome. J. Neuropathol. Exp. Neurol. 48, 270-289.
24. Gressens, P., Gofflot, F., Van Maele-Fabry, G., Misson, J.-P., Gadisseux, J.-F., Evrard, P., and Picard, J. J. (1992) Early neurogenesis and teratogenesis in whole mouse embryo cultures. Histochemical, immunocytological and ultrastructural study of the premigratory neuronal-glial units in normal mouse embryo and in mouse embryos influenced by cocaine and retinoic acid. J. Neuropathol. Exp. Neurol. 51, 206-219.
25. Fan, C.-Y., Pan, J., Chu, R., Lee, D., Kluckman, D., Usuda, N., et al. (1996) Hepatocellular and hepatic peroxisomal alterations in mice with a disrupted peroxisomal fatty acylcoenzyme A oxidase gene. J. Biol. Chem. 271, 24,698-24,710.
26. Seedorf, U., Raabe, M., Ellinghaus, P., Kannenberg, F., Fobker, M., Engel, T., et al. (1998) Defective peroxisomal catabolism of branched fatty acyl coenzyme A in mice lacking the sterol carrier protein-2/sterol carrier protein-x gene function. Genes Dev. 12, 1189-1201.