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

Volume: 129, Issue: 3, Pages: 399 - 415
Published: Dec 31, 2014
Abstract
X-linked adrenoleukodystrophy (X-ALD) is a rare neurometabolic disease characterized by the accumulation of very long chain fatty acids (VLCFAs) due to a loss of function of the peroxisomal transporter ABCD1. Here, using in vivo and in vitro models, we demonstrate that autophagic flux was impaired due to elevated mammalian target of rapamycin (mTOR) signaling, which contributed to X-ALD pathogenesis. We also show that excess VLCFAs downregulated...
Paper Details
Title
Autophagy induction halts axonal degeneration in a mouse model of X-adrenoleukodystrophy
Published Date
Dec 31, 2014
Volume
129
Issue
3
Pages
399 - 415
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