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Items: 1 to 20 of 38


A Thyroid Hormone-Based Strategy for Correcting the Biochemical Abnormality in X-Linked Adrenoleukodystrophy.

Hartley MD, Kirkemo LL, Banerji T, Scanlan TS.

Endocrinology. 2017 May 1;158(5):1328-1338. doi: 10.1210/en.2016-1842.


Tauroursodeoxycholic bile acid arrests axonal degeneration by inhibiting the unfolded protein response in X-linked adrenoleukodystrophy.

Launay N, Ruiz M, Grau L, Ortega FJ, Ilieva EV, Martínez JJ, Galea E, Ferrer I, Knecht E, Pujol A, Fourcade S.

Acta Neuropathol. 2017 Feb;133(2):283-301. doi: 10.1007/s00401-016-1655-9. Epub 2016 Dec 21.


C26:0-Carnitine Is a New Biomarker for X-Linked Adrenoleukodystrophy in Mice and Man.

van de Beek MC, Dijkstra IM, van Lenthe H, Ofman R, Goldhaber-Pasillas D, Schauer N, Schackmann M, Engelen-Lee JY, Vaz FM, Kulik W, Wanders RJ, Engelen M, Kemp S.

PLoS One. 2016 Apr 28;11(4):e0154597. doi: 10.1371/journal.pone.0154597. eCollection 2016.


Peroxisomes in brain development and function.

Berger J, Dorninger F, Forss-Petter S, Kunze M.

Biochim Biophys Acta. 2016 May;1863(5):934-55. doi: 10.1016/j.bbamcr.2015.12.005. Epub 2015 Dec 11. Review.


Functional Characterization of IPSC-Derived Brain Cells as a Model for X-Linked Adrenoleukodystrophy.

Baarine M, Khan M, Singh A, Singh I.

PLoS One. 2015 Nov 18;10(11):e0143238. doi: 10.1371/journal.pone.0143238. eCollection 2015.


Brain endothelial dysfunction in cerebral adrenoleukodystrophy.

Musolino PL, Gong Y, Snyder JM, Jimenez S, Lok J, Lo EH, Moser AB, Grabowski EF, Frosch MP, Eichler FS.

Brain. 2015 Nov;138(Pt 11):3206-20. doi: 10.1093/brain/awv250. Epub 2015 Sep 15.


Redox interplay between mitochondria and peroxisomes.

Lismont C, Nordgren M, Van Veldhoven PP, Fransen M.

Front Cell Dev Biol. 2015 May 27;3:35. doi: 10.3389/fcell.2015.00035. eCollection 2015. Review.


Activation of sirtuin 1 as therapy for the peroxisomal disease adrenoleukodystrophy.

Morató L, Ruiz M, Boada J, Calingasan NY, Galino J, Guilera C, Jové M, Naudí A, Ferrer I, Pamplona R, Serrano M, Portero-Otín M, Beal MF, Fourcade S, Pujol A.

Cell Death Differ. 2015 Nov;22(11):1742-53. doi: 10.1038/cdd.2015.20. Epub 2015 Mar 27.


Adenoassociated virus serotype 9-mediated gene therapy for x-linked adrenoleukodystrophy.

Gong Y, Mu D, Prabhakar S, Moser A, Musolino P, Ren J, Breakefield XO, Maguire CA, Eichler FS.

Mol Ther. 2015 May;23(5):824-34. doi: 10.1038/mt.2015.6. Epub 2015 Jan 16.


Autophagy induction halts axonal degeneration in a mouse model of X-adrenoleukodystrophy.

Launay N, Aguado C, Fourcade S, Ruiz M, Grau L, Riera J, Guilera C, Giròs M, Ferrer I, Knecht E, Pujol A.

Acta Neuropathol. 2015 Mar;129(3):399-415. doi: 10.1007/s00401-014-1378-8. Epub 2014 Dec 31.


ABCD1 deletion-induced mitochondrial dysfunction is corrected by SAHA: implication for adrenoleukodystrophy.

Baarine M, Beeson C, Singh A, Singh I.

J Neurochem. 2015 May;133(3):380-96. doi: 10.1111/jnc.12992. Epub 2015 Jan 13.


Abcd2 is a strong modifier of the metabolic impairments in peritoneal macrophages of ABCD1-deficient mice.

Muneer Z, Wiesinger C, Voigtländer T, Werner HB, Berger J, Forss-Petter S.

PLoS One. 2014 Sep 25;9(9):e108655. doi: 10.1371/journal.pone.0108655. eCollection 2014.


Oxidative stress and adrenocortical insufficiency.

Prasad R, Kowalczyk JC, Meimaridou E, Storr HL, Metherell LA.

J Endocrinol. 2014 Jun;221(3):R63-73. doi: 10.1530/JOE-13-0346. Epub 2014 Mar 12. Review.


Pathophysiology of X-linked adrenoleukodystrophy.

Berger J, Forss-Petter S, Eichler FS.

Biochimie. 2014 Mar;98:135-42. doi: 10.1016/j.biochi.2013.11.023. Epub 2013 Dec 4. Review.


Histone deacetylase inhibitor upregulates peroxisomal fatty acid oxidation and inhibits apoptotic cell death in abcd1-deficient glial cells.

Singh J, Khan M, Pujol A, Baarine M, Singh I.

PLoS One. 2013 Jul 26;8(7):e70712. doi: 10.1371/journal.pone.0070712. Print 2013.


Pioglitazone halts axonal degeneration in a mouse model of X-linked adrenoleukodystrophy.

Morató L, Galino J, Ruiz M, Calingasan NY, Starkov AA, Dumont M, Naudí A, Martínez JJ, Aubourg P, Portero-Otín M, Pamplona R, Galea E, Beal MF, Ferrer I, Fourcade S, Pujol A.

Brain. 2013 Aug;136(Pt 8):2432-43. doi: 10.1093/brain/awt143. Epub 2013 Jun 22.


Glutathione imbalance in patients with X-linked adrenoleukodystrophy.

Petrillo S, Piemonte F, Pastore A, Tozzi G, Aiello C, Pujol A, Cappa M, Bertini E.

Mol Genet Metab. 2013 Aug;109(4):366-70. doi: 10.1016/j.ymgme.2013.05.009. Epub 2013 May 22.


Oxidative stress modulates mitochondrial failure and cyclophilin D function in X-linked adrenoleukodystrophy.

López-Erauskin J, Galino J, Bianchi P, Fourcade S, Andreu AL, Ferrer I, Muñoz-Pinedo C, Pujol A.

Brain. 2012 Dec;135(Pt 12):3584-98. doi: 10.1093/brain/aws292.


The gene expression profiles of induced pluripotent stem cells from individuals with childhood cerebral adrenoleukodystrophy are consistent with proposed mechanisms of pathogenesis.

Wang XM, Yik WY, Zhang P, Lu W, Dranchak PK, Shibata D, Steinberg SJ, Hacia JG.

Stem Cell Res Ther. 2012 Oct 4;3(5):39. doi: 10.1186/scrt130.

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