Neurometabolic Diseases Lab

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21 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 346
22 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):92 330
23 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 224
24 Activation of sirtuin 1 as therapy for the peroxisomal disease adrenoleukodystrophy. Cell Death Differ. 2015 Mar 27. doi: 10.1038/cdd.2015.20 352
25 Autophagy induction halts axonal degeneration in a mouse model of X-adrenoleukodystrophy. Acta Neuropathol. 2014 Dec 31. [Epub ahead of print] 442
26 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. 348
27 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 1 297
28 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] 362
29 Mitochondrial dysfunction in central nervous system white matter disorders.Glia. 2014 May 28. doi: 10.1002/glia.22670 385
30 Pharmacological Inhibition of Poly(ADP-Ribose) Polymerases Improves Fitness and Mitochondrial Function in Skeletal Muscle. Cell Metab. 2014 June; 19, 1-8 394
31 PDR-1/hParkin negatively regulates the phagocytosis of apoptotic cell corpses in Caenorhabditis elegans. Cell Death Dis. 2014 Mar 13;5:e1120. 360
32 Methylene blue upregulates Nrf2/ARE genes and prevents tau-related neurotoxicity. Hum Mol Genet. 2014 Mar 3. doi:10.1093/hmg/ddu080 448
33 PGC-1α overexpression exacerbates β-amyloid and tau deposition in a transgenic mouse model of Alzheimer's disease. FASEB J. 2014 Apr;28(4):1745-55 379
34 Mitochondrial dysfunction and oxidative damage cooperatively fuel axonal degeneration in X-linked adrenoleukodystrophy. Biochimie. 2014 Mar;98:143-9 570
35 Pioglitazone halts axonal degeneration in a mouse model of X-linked adrenoleukodystrophy. Brain. 2013 Aug;136(Pt 8):2432-43. 1068
36 Impaired mitochondrial oxidative phosphorylation in the peroxisomal disease X-linked adrenoleukodystrophy. Hum Mol Genet. 2013 Aug 15;22(16):3296-305 1158
37 Glutathione imbalance in patients with X-linked adrenoleukodystrophy. Mol Genet Metab. 2013 Aug;109(4):366-70. 362
38 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 354
39 Functional Genomics Reveals Dysregulation of Cortical Olfactory Receptors in Parkinson Disease: Novel Putative Chemoreceptors in the Human Brain. J Neuropathol Exp Neurol. 2013 June 611
40 Cyclophilin D as a potential target for antioxidants in neurodegeneration: the X-ALD case. Biol Chem. 2013 May 1;394(5):621-9 578
 
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