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145 results

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Page 1
The peroxisome deficient PEX2 Zellweger mouse: pathologic and biochemical correlates of lipid dysfunction.
Faust PL, Su HM, Moser A, Moser HW. Faust PL, et al. J Mol Neurosci. 2001 Apr-Jun;16(2-3):289-97; discussion 317-21. doi: 10.1385/JMN:16:2-3:289. J Mol Neurosci. 2001. PMID: 11478384 Review.
A murine model for this disorder was previously developed by targeted deletion of the PEX2 peroxisomal gene. By labeling neuronal precursor cells in vivo with a mitotic marker, we can demonstrate a delay in neuronal migration in the cerebral cortex of homozygous PEX2 …
A murine model for this disorder was previously developed by targeted deletion of the PEX2 peroxisomal gene. By labeling neuronal pre …
Peroxisomal β-oxidation acts as a sensor for intracellular fatty acids and regulates lipolysis.
Ding L, Sun W, Balaz M, He A, Klug M, Wieland S, Caiazzo R, Raverdy V, Pattou F, Lefebvre P, Lodhi IJ, Staels B, Heim M, Wolfrum C. Ding L, et al. Nat Metab. 2021 Dec;3(12):1648-1661. doi: 10.1038/s42255-021-00489-2. Epub 2021 Dec 13. Nat Metab. 2021. PMID: 34903883 Free PMC article.
Here we report a sensing mechanism for cellular FAs based on peroxisomal degradation of FAs and coupled with reactive oxygen species (ROS) production, which in turn regulates FA release by modulating lipolysis. Changes in ROS levels are sensed by PEX2, which modulates ATGL …
Here we report a sensing mechanism for cellular FAs based on peroxisomal degradation of FAs and coupled with reactive oxygen species (ROS) p …
PEX2 is the E3 ubiquitin ligase required for pexophagy during starvation.
Sargent G, van Zutphen T, Shatseva T, Zhang L, Di Giovanni V, Bandsma R, Kim PK. Sargent G, et al. J Cell Biol. 2016 Sep 12;214(6):677-90. doi: 10.1083/jcb.201511034. Epub 2016 Sep 5. J Cell Biol. 2016. PMID: 27597759 Free PMC article.
Here, we report that the peroxisomal E3 ubiquitin ligase peroxin 2 (PEX2) is the causative agent for mammalian pexophagy. Expression of PEX2 leads to gross ubiquitination of peroxisomes and degradation of peroxisomes in an NBR1-dependent autophagic process. We ident …
Here, we report that the peroxisomal E3 ubiquitin ligase peroxin 2 (PEX2) is the causative agent for mammalian pexophagy. Expression …
Zellweger Spectrum Disorder.
Steinberg SJ, Raymond GV, Braverman NE, Moser AB. Steinberg SJ, et al. 2003 Dec 12 [updated 2020 Oct 29]. In: Adam MP, Feldman J, Mirzaa GM, Pagon RA, Wallace SE, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2025. 2003 Dec 12 [updated 2020 Oct 29]. In: Adam MP, Feldman J, Mirzaa GM, Pagon RA, Wallace SE, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2025. PMID: 20301621 Free Books & Documents. Review.
Restoration of PEX2 peroxisome assembly defects by overexpression of PMP70.
Gärtner J, Brosius U, Obie C, Watkins PA, Valle D. Gärtner J, et al. Eur J Cell Biol. 1998 Aug;76(4):237-45. doi: 10.1016/S0171-9335(98)80001-0. Eur J Cell Biol. 1998. PMID: 9765053
By contrast, expression of a mutant allele of PMP70 identified in a patient with Zellweger syndrome did not restore peroxisome biogenesis in the PEX2-deficient CHO cell clones. Our results indicate that overexpression of PMP70 suppresses the phenotype of PEX2 gene m …
By contrast, expression of a mutant allele of PMP70 identified in a patient with Zellweger syndrome did not restore peroxisome biogenesis in …
Disturbed cholesterol homeostasis in a peroxisome-deficient PEX2 knockout mouse model.
Kovacs WJ, Shackelford JE, Tape KN, Richards MJ, Faust PL, Fliesler SJ, Krisans SK. Kovacs WJ, et al. Mol Cell Biol. 2004 Jan;24(1):1-13. doi: 10.1128/MCB.24.1.1-13.2004. Mol Cell Biol. 2004. PMID: 14673138 Free PMC article.
Compared with wild-type mice, PEX2(-/-) mice have decreased total and high-density lipoprotein cholesterol levels in plasma. ...The classic pathway of bile acid biosynthesis is downregulated in PEX2(-/-) mice. However, expression of CYP27A1, the rate-determining enz …
Compared with wild-type mice, PEX2(-/-) mice have decreased total and high-density lipoprotein cholesterol levels in plasma. ...The c …
Pex2 and pex12 function as protein-ubiquitin ligases in peroxisomal protein import.
Platta HW, El Magraoui F, Bäumer BE, Schlee D, Girzalsky W, Erdmann R. Platta HW, et al. Mol Cell Biol. 2009 Oct;29(20):5505-16. doi: 10.1128/MCB.00388-09. Epub 2009 Aug 17. Mol Cell Biol. 2009. PMID: 19687296 Free PMC article.
It is demonstrated that each of the three RING peroxins Pex2, Pex10, and Pex12 exhibits ubiquitin-protein isopeptide ligase activity. Our results show that Pex2 mediates the Ubc4-dependent polyubiquitination whereas Pex12 facilitates the Pex4-dependent monoubiquitin …
It is demonstrated that each of the three RING peroxins Pex2, Pex10, and Pex12 exhibits ubiquitin-protein isopeptide ligase activity. …
Peroxisomes and photomorphogenesis.
Kaur N, Li J, Hu J. Kaur N, et al. Subcell Biochem. 2013;69:195-211. doi: 10.1007/978-94-007-6889-5_11. Subcell Biochem. 2013. PMID: 23821150 Review.
Lastly, a gain-of-function mutant of the peroxisomal membrane-tethered RING-type E3 ubiquitin ligase PEX2 partially suppresses the phenotype of the photomorphogenic mutant det1. Possible mechanisms underlying this phenomenon are discussed....
Lastly, a gain-of-function mutant of the peroxisomal membrane-tethered RING-type E3 ubiquitin ligase PEX2 partially suppresses the ph …
Cholesterol biosynthesis and ER stress in peroxisome deficiency.
Faust PL, Kovacs WJ. Faust PL, et al. Biochimie. 2014 Mar;98:75-85. doi: 10.1016/j.biochi.2013.10.019. Epub 2013 Nov 7. Biochimie. 2014. PMID: 24211592 Review.
This review intends to highlight recent findings illustrating the important role peroxisomes play in cholesterol biosynthesis and maintenance of cholesterol homeostasis. Disruption of the Pex2 gene leads to peroxisome deficiency and widespread metabolic dysfunction. The …
This review intends to highlight recent findings illustrating the important role peroxisomes play in cholesterol biosynthesis and maintenanc …
The peroxisomal import proteins PEX2, PEX5 and PEX7 are differently involved in Podospora anserina sexual cycle.
Bonnet C, Espagne E, Zickler D, Boisnard S, Bourdais A, Berteaux-Lecellier V. Bonnet C, et al. Mol Microbiol. 2006 Oct;62(1):157-69. doi: 10.1111/j.1365-2958.2006.05353.x. Mol Microbiol. 2006. PMID: 16987176 Free article.
PEX2 belongs to the RING-finger complex located in the peroxisomal membrane and acts in protein import downstream of PEX5 and PEX7; it is therefore required for the import of both PTS1 and PTS2 proteins. We have shown previously that PEX2 deficiency leads to an impa …
PEX2 belongs to the RING-finger complex located in the peroxisomal membrane and acts in protein import downstream of PEX5 and PEX7; i …
145 results