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2. Cholesterol biosynthesis and ER stress in peroxisome deficiency. Faust PL, Kovacs WJ. Biochimie; 2014 Mar; 98():75-85. PubMed ID: 24211592 [Abstract] [Full Text] [Related]
3. Peroxisome deficiency-induced ER stress and SREBP-2 pathway activation in the liver of newborn PEX2 knock-out mice. Kovacs WJ, Charles KN, Walter KM, Shackelford JE, Wikander TM, Richards MJ, Fliesler SJ, Krisans SK, Faust PL. Biochim Biophys Acta; 2012 Jun; 1821(6):895-907. PubMed ID: 22441164 [Abstract] [Full Text] [Related]
4. The peroxisome deficient PEX2 Zellweger mouse: pathologic and biochemical correlates of lipid dysfunction. Faust PL, Su HM, Moser A, Moser HW. J Mol Neurosci; 2001 Jun; 16(2-3):289-97; discussion 317-21. PubMed ID: 11478384 [Abstract] [Full Text] [Related]
5. Peroxisome deficiency causes a complex phenotype because of hepatic SREBP/Insig dysregulation associated with endoplasmic reticulum stress. Kovacs WJ, Tape KN, Shackelford JE, Wikander TM, Richards MJ, Fliesler SJ, Krisans SK, Faust PL. J Biol Chem; 2009 Mar 13; 284(11):7232-45. PubMed ID: 19110480 [Abstract] [Full Text] [Related]
6. Bile acid treatment alters hepatic disease and bile acid transport in peroxisome-deficient PEX2 Zellweger mice. Keane MH, Overmars H, Wikander TM, Ferdinandusse S, Duran M, Wanders RJ, Faust PL. Hepatology; 2007 Apr 13; 45(4):982-97. PubMed ID: 17393522 [Abstract] [Full Text] [Related]
7. Targeted deletion of the PEX2 peroxisome assembly gene in mice provides a model for Zellweger syndrome, a human neuronal migration disorder. Faust PL, Hatten ME. J Cell Biol; 1997 Dec 01; 139(5):1293-305. PubMed ID: 9382874 [Abstract] [Full Text] [Related]
9. PEX11 beta deficiency is lethal and impairs neuronal migration but does not abrogate peroxisome function. Li X, Baumgart E, Morrell JC, Jimenez-Sanchez G, Valle D, Gould SJ. Mol Cell Biol; 2002 Jun 01; 22(12):4358-65. PubMed ID: 12024045 [Abstract] [Full Text] [Related]
10. Pex13 inactivation in the mouse disrupts peroxisome biogenesis and leads to a Zellweger syndrome phenotype. Maxwell M, Bjorkman J, Nguyen T, Sharp P, Finnie J, Paterson C, Tonks I, Paton BC, Kay GF, Crane DI. Mol Cell Biol; 2003 Aug 01; 23(16):5947-57. PubMed ID: 12897163 [Abstract] [Full Text] [Related]
11. Restoration of PEX2 peroxisome assembly defects by overexpression of PMP70. Gärtner J, Brosius U, Obie C, Watkins PA, Valle D. Eur J Cell Biol; 1998 Aug 01; 76(4):237-45. PubMed ID: 9765053 [Abstract] [Full Text] [Related]
12. Functional Peroxisomes Are Essential for Efficient Cholesterol Sensing and Synthesis. Charles KN, Shackelford JE, Faust PL, Fliesler SJ, Stangl H, Kovacs WJ. Front Cell Dev Biol; 2020 Aug 01; 8():560266. PubMed ID: 33240873 [Abstract] [Full Text] [Related]
13. Peroxisome biogenesis disorders: the role of peroxisomes and metabolic dysfunction in developing brain. Faust PL, Banka D, Siriratsivawong R, Ng VG, Wikander TM. J Inherit Metab Dis; 2005 Aug 01; 28(3):369-83. PubMed ID: 15868469 [Abstract] [Full Text] [Related]
14. Overexpression of a human and a fungal ABC transporter similarly suppresses the differentiation defects of a fungal peroxisomal mutant but introduces pleiotropic cellular effects. Boisnard S, Zickler D, Picard M, Berteaux-Lecellier V. Mol Microbiol; 2003 Sep 01; 49(5):1287-96. PubMed ID: 12940987 [Abstract] [Full Text] [Related]
15. Failure of microtubule-mediated peroxisome division and trafficking in disorders with reduced peroxisome abundance. Nguyen T, Bjorkman J, Paton BC, Crane DI. J Cell Sci; 2006 Feb 15; 119(Pt 4):636-45. PubMed ID: 16449325 [Abstract] [Full Text] [Related]
16. Abnormal cerebellar histogenesis in PEX2 Zellweger mice reflects multiple neuronal defects induced by peroxisome deficiency. Faust PL. J Comp Neurol; 2003 Jun 30; 461(3):394-413. PubMed ID: 12746876 [Abstract] [Full Text] [Related]
17. Different accumulations of 3-ketoacyl-CoA thiolase precursor in peroxisomes of Chinese hamster ovary cells harboring a dysfunction in the PEX2 protein. Huang Y, Ito R, Imanaka T, Usuda N, Ito M. Biochim Biophys Acta; 2002 May 08; 1589(3):273-84. PubMed ID: 12031794 [Abstract] [Full Text] [Related]
18. Absence of functional peroxisomes does not lead to deficiency of enzymes involved in cholesterol biosynthesis. Hogenboom S, Romeijn GJ, Houten SM, Baes M, Wanders RJ, Waterham HR. J Lipid Res; 2002 Jan 08; 43(1):90-8. PubMed ID: 11792727 [Abstract] [Full Text] [Related]
19. Drosophila carrying pex3 or pex16 mutations are models of Zellweger syndrome that reflect its symptoms associated with the absence of peroxisomes. Nakayama M, Sato H, Okuda T, Fujisawa N, Kono N, Arai H, Suzuki E, Umeda M, Ishikawa HO, Matsuno K. PLoS One; 2011 Jan 08; 6(8):e22984. PubMed ID: 21826223 [Abstract] [Full Text] [Related]