Peroxisome biogenesis disorders: The role of peroxisomes and metabolic dysfunction in developing brain

Journal of Inherited Metabolic Disease

Volumn 28, Issue 3, 2005, Pages 369-383

  Faust, P L ^a   Banka, D ^a   Siriratsivawong, R ^a   Ng, V G ^a   Wikander, T M ^a  

^a Howard Hughes Medical Institute   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

BILE ACID;

ARTICLE; BIOGENESIS; BRAIN CELL; BRAIN CORTEX; BRAIN DEVELOPMENT; CELL CULTURE; CELL DIFFERENTIATION; CELL GROWTH; CELL MIGRATION; CELL PROLIFERATION; CELL SURVIVAL; CENTRAL NERVOUS SYSTEM; CEREBELLUM; DISEASE ASSOCIATION; DISEASE MODEL; DISEASE SEVERITY; IN VITRO STUDY; METABOLIC ENCEPHALOPATHY; MOUSE; NERVE CELL; NERVOUS SYSTEM FUNCTION; NERVOUS SYSTEM MALFORMATION; NONHUMAN; PATHOGENESIS; PEROXISOME; PHENOTYPE; ZELLWEGER SYNDROME;

ANIMALS; BRAIN; BRAIN CHEMISTRY; CELL DIFFERENTIATION; CELL MOVEMENT; HUMANS; METABOLISM, INBORN ERRORS; MICE; MICE, KNOCKOUT; PEROXISOMAL DISORDERS; PEROXISOMES; PURKINJE CELLS; ZELLWEGER SYNDROME;

EID: 19444371845     PISSN: 01418955     EISSN: None     Source Type: Journal    
DOI: 10.1007/s10545-005-7059-y     Document Type: Article

References (38)

1. Altman J, Bayer S (1997) Development of the Cerebellar System. New York: CRC Press.
2. Arnold G, Holtzman E (1978) Microperoxisomes in the central nervous system of the postnatal rat. Brain Res 155: 1-17.
3. Baes M, Gressens P, Baumgart E, et al (1997) A mouse model for Zellweger syndrome. Nature Genet 17: 49-57.
4. Baes M, Gressens P, Huyghe S, et al (2002) The neuronal migration defect in mice with Zellweger syndrome (Pex5 knockout) is not caused by the inactivity of peroxisomal beta-oxidation. J Neuropathol Exp Neurol 61: 368-374.
5. Baptista CA, Hatten ME, Blazeski R, Mason CA (1994) Cell-cell interactions influence survival and differentiation of purified Purkinje cells in vitro. Neuron 12: 243-260.
6. Bradke F, Dotti CG (1997) Neuronal polarity: Vectorial cytoplasmic flow precedes axon formation. Neuron 19: 1175-1186.
7. Dobyns WB, Truwit CL (1995) Lissencephaly and other malformations of cortical development: 1995 update. Neuropediatrics 26: 132-147.
8. Dusart I, Airaksinen MS, Sotelo C (1997) Purkinje cell survival and axonal regeneration are age dependent: An in vitro study. J Neurosci 17: 3710-3726.
9. Edmondson JC, Hatten ME (1987) Glial-guided granule neuron migration in vitro: A high-resolution time-lapse video microscopic study. J Neurosci 7: 1928-1934.
10. Evrard P, Caviness VS, Prats-Vinas J, Lyon G (1978) The mechanism of arrest of neuronal migration in the Zellweger malformation: An hypothesis based on cytoarchitectonic analysis. Acta Neuropathol (Berl) 41: 109-117.
11. Faust (2003) Abnormal cerebellar histogenesis in PEX2 Zellweger mice reflects multiple neuronal defects induced by peroxisome deficiency. J Comp Neurol 461: 394-413.
12. Faust PL, Hatten ME (1997) Targeted deletion of the PEX2 peroxisome assambly gene in mice provides a model for Zellweger syndrome, a human neuronal migration disorder. J Cell Biol 139: 1293-1305.
13. Faust PL, Su H-M, Moser A, Moser HW (2001) The peroxisome deficient PEX2 Zellweger mouse: Pathologic and biochemical correlates of lipid dysfunction. J Mol Neurosci 16: 289-297.
14. Fujiki Y (2000) Peroxisome biogenesis and peroxisome biogenesis disorders. FEBS Lett 476: 42-46.
15. Gould SJ, Valle D (2000) Peroxisome biogenesis disorders: Genetics and cell biology. Trends Genet 16: 340-345.
16. Gould SJ, Raymond GV, Valle D (2001) The peroxisome biogenesis disorders. In Scriver CR, Beaudet AL, Sly WS, Valle D, eds; Childs B, Kinzler KW, Vogelstein B, assoc. eds. The Metabolic and Molecular Bases of Inherited Disease, 8th edn. New York: McGraw-Hill, 3181-3217.
17. Hatten ME, Mason CA (1990) Mechanisms of neural patterning and specification in the developing cerebellum. Annu Rev Neurosci 18: 385-408.
18. Heuer H, Mason CA (2003) Thyroid hormone induces cerebellar Purkinje cell dendritic development via the thyroid hormone receptor alpha1. J Neurosci 23: 10604-10612.
19. Ito M (1984) Purkinje cells: Abnormal morphology. The Cerebellum and Neural Control. New York: Raven Press, 40-50.
20. Janssen A, Baes M, Gressens P, Mannaerts GP, Declercq P, Van Veldhoven PP (2000) Docosahexaenoic acid deficit is not a major pathogenic factor in peroxisome-deficient mice. Lab Invest 80: 31-35.
21. Janssen A, Gressens P, Grabenbauer M, et al (2003) Neuronal migration depends on intact peroxisomal function in brain and in extraneuronal tissues. J Neurosci 23: 9732-9741.
22. Kaufmann WE, Theda C, Naidu S, Watkins PA, Moser AB, Moser HW (1996) Neuronal migration abnormality in peroxisomal bifunctional enzyme defect. Ann Neurol 39: 268-271.
23. Li X, Baumgart E, Morrell JC, Jimenez-Sanchez G, Valle D, Gould SJ (2002) PEX11 beta deficiency is lethal and impairs neuronal migration but does not abrogate peroxisome function. Mol Cell Biol 22: 4358-4365.
24. Maeda K, Kimura A, Yamato Y, et al (2002) Oral bile acid treatment in two Japanese patients with Zellweger syndrome. J Pediatr Gastroenterol Nutr 35: 227-230.
25. Maxwell M, Bjorkman J, Nguyen T, et al (2003) PEX13 inactivation in the mouse disrupts peroxisome biogenesis and leads to a Zellweger phenotype. Mol Cell Biol 23: 5957-5957.
26. McKenna O, Arnold G, Holtzman E (1976) Microperoxisome distribution in the central nervous system of the rat. Brain Res 117: 181-194.
27. Opitz JM, ZuRhein GM, Vitale L, et al (1969) The Zellweger syndrome (cerebro-hepato-renal syndrome). Birth Defects 5: 144-158.
28. Powers JM, Moser, HW, Moser AB, et al (1985) Fetal cerebrohepatorenal (Zellweger) syndrome: Dysmorphic, radiologic, biochemical and pathologic findings in four affected fetuses. Hum Pathol 16: 610-620.
29. Powers JM, Moser HW (1998) Peroxisomal disorders: Genotype, phenotype, major neuropathologic lesions, and pathogenesis. Brain Pathol 8: 101-120.
30. Powers JM, Tummons RC, Caviness VC Jr, Moser AB, Moser HW (1989) Structural and chemical alterations in the cerebral maldevelopment of fetal cerebro-hepato-renal (Zellweger) syndrome. J Neuropathol Exp Neurol 48: 270-289.
31. Rakic P (1971) Nueron-glia relationship during granule cell migration in developing cerebellar cortex. A Golgi and electron microscopic study in Macacus rhesus. J Comp Neurol 141: 283-312.
32. Rivas RJ, Hatten ME (1995) Motility and cytoskeletal organization of migrating cerebellar granule neurons. J Neurosci 15: 981-989.
33. Ross ME, Walsh CA (2001) Human brain malformations and their lessons for neuronal migration. Annu Rev Neurosci 24: 1041-1070.
34. Russell DW (1999) Nuclear orphan receptors control cholesterol catabolism. Cell 97: 539-542.
35. Setchell K, Bragetti P, Zimmer-Nechemias L, et al (1992) Oral bile acid treatment and the patient with Zellweger syndrome. Hepatology 15: 198-207.
36. Volpe JJ, Adams RD (1972) Cerebro-hepato-renal syndrome of Zellweger: An inherited disorder of neuronal migration. Acta Neuropathol (Berl) 20: 175-198.
37. Wanders RJA, Tager JM (1998) Lipid metabolism in peroxisomes in relation to human disease. Mol Aspects Med 19: 69-154.
38. Wanders RJA, Barth PG, Heymans HAS (2001) Single peroxisomal enzyme deficiencies. In: Scriver CR, Beaudet AL, Sly WS, Valle D, eds; Childs B, Kinzler KW, Vogelstein B, assoc eds. The Metabolic and Molecular Bases of Inherited Disease, 8th edn. New York: McGraw-Hill, 3219-3256.