Neurometabolic Diseases Lab

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21 Oxidative stress, mitochondrial and proteostasis malfunction in adrenoleukodystrophy: A paradigm for axonal degeneration. Free Radic Biol Med. 2015 Jun 12. pii: S0891-5849(15)00264-6 371
22 Mitochondrial Disease Sequence Data Resource (MSeqDR): a global grass-roots consortium to facilitate deposition, curation, annotation, and integrated analysis of genomic data for the mitochondrial disease clinical and research communities. Mol Genet Metab 366
23 Astrocytes and mitochondria from adrenoleukodystrophy protein (ABCD1)-deficient mice reveal that the adrenoleukodystrophy-associated very long-chain fatty acids target several cellular energy-dependent functions. Biochim Biophys Acta. 2015 May;1852(5):92 349
24 Neuroinflammatory signals in Alzheimer disease and APP/PS1 transgenic mice: correlations with plaques, tangles, and oligomeric species. Journal of Neuropathology and Experimental Neurology. 2015 Apr 241
25 Activation of sirtuin 1 as therapy for the peroxisomal disease adrenoleukodystrophy. Cell Death Differ. 2015 Mar 27. doi: 10.1038/cdd.2015.20 368
26 Autophagy induction halts axonal degeneration in a mouse model of X-adrenoleukodystrophy. Acta Neuropathol. 2014 Dec 31. [Epub ahead of print] 463
27 Deregulation of purine metabolism in Alzheimer's disease. Neurobiol Aging. 2015 Jan;36(1):68-80. doi: 10.1016/j.neurobiolaging.2014.08.004. Epub 2014 Aug 8. 365
28 ABCD2 alters peroxisome proliferator-activated receptor a signaling in vitro, but does not impair responses to fenofibrate therapy in a mouse model of diet-induced obesity. Mol Pharmacol. 2014 Nov;86(5):505-13. doi: 10.1124/mol.114.092742. Epub 2014 Aug 1 315
29 Improvement of the Rett Syndrome Phenotype in a Mecp2 Mouse Model Upon Treatment with Levodopa and a Dopa Decarboxylase Inhibitor. Neuropsychopharmacology. 2014 Jun 11. doi: 10.1038/npp.2014.136. [Epub ahead of print] 386
30 Mitochondrial dysfunction in central nervous system white matter disorders.Glia. 2014 May 28. doi: 10.1002/glia.22670 402
31 Pharmacological Inhibition of Poly(ADP-Ribose) Polymerases Improves Fitness and Mitochondrial Function in Skeletal Muscle. Cell Metab. 2014 June; 19, 1-8 410
32 PDR-1/hParkin negatively regulates the phagocytosis of apoptotic cell corpses in Caenorhabditis elegans. Cell Death Dis. 2014 Mar 13;5:e1120. 378
33 Methylene blue upregulates Nrf2/ARE genes and prevents tau-related neurotoxicity. Hum Mol Genet. 2014 Mar 3. doi:10.1093/hmg/ddu080 462
34 PGC-1α overexpression exacerbates β-amyloid and tau deposition in a transgenic mouse model of Alzheimer's disease. FASEB J. 2014 Apr;28(4):1745-55 393
35 Mitochondrial dysfunction and oxidative damage cooperatively fuel axonal degeneration in X-linked adrenoleukodystrophy. Biochimie. 2014 Mar;98:143-9 586
36 Pioglitazone halts axonal degeneration in a mouse model of X-linked adrenoleukodystrophy. Brain. 2013 Aug;136(Pt 8):2432-43. 1091
37 Impaired mitochondrial oxidative phosphorylation in the peroxisomal disease X-linked adrenoleukodystrophy. Hum Mol Genet. 2013 Aug 15;22(16):3296-305 1187
38 Glutathione imbalance in patients with X-linked adrenoleukodystrophy. Mol Genet Metab. 2013 Aug;109(4):366-70. 373
39 Histone deacetylase inhibitor upregulates peroxisomal fatty acid oxidation and inhibits apoptotic cell death in abcd1-deficient glial cells. PLoS One. 2013 Jul 26;8(7):e70712 364
40 Functional Genomics Reveals Dysregulation of Cortical Olfactory Receptors in Parkinson Disease: Novel Putative Chemoreceptors in the Human Brain. J Neuropathol Exp Neurol. 2013 June 623
 
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