Browsing Medicina Experimental by Subject "Adrenoleukodystrophy"

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Aberrant regulation of the GSK-3b/NRF2 axis unveils a novel therapy for adrenoleukodystrophy

Ranea-Robles, Pablo; Launay, Nathalie; Ruiz, Montserrat; Calingasan, Noel Ylagan; Dumont, Magali; Naudí i Farré, Alba; Portero Otín, Manuel; Pamplona Gras, Reinald; Ferrer, Isidre; Beal, M. Flint; Fourcade, Stéphane; Pujol, Aurora (Wiley Open Access, 2018)

The nuclear factor erythroid 2-like 2 (NRF2) is the master regulator of endogenous antioxidant responses. Oxidative damage is a shared and early-appearing feature in X-linked adrenoleukodystrophy (X-ALD) patients and ...
Oxidative stress underlying axonal degeneration in adrenoleukodystrophy: a paradigm for multifactorial neurodegenerative diseases?

Galea, Elena; Launay, Nathalie; Portero Otín, Manuel; Ruiz, Montserrat; Pamplona Gras, Reinald; Aubourg, Patrick; Ferrer, Isidre; Pujol, Aurora (Elsevier, 2012)

X-linked adrenoleukodystrophy (X-ALD) is an inherited neurodegenerative disorder expressed as four disease variants characterized by adrenal insufficiency and graded damage in the nervous system. X-ALD is caused by a loss ...