作者
Jone López‐Erauskin, Stephane Fourcade, Jorge Galino, Montserrat Ruiz, Agatha Schlüter, Alba Naudi, Mariona Jove, Manuel Portero‐Otin, Reinald Pamplona, Isidre Ferrer, Aurora Pujol
发表日期
2011/7
期刊
Annals of neurology
卷号
70
期号
1
页码范围
84-92
出版商
Wiley Subscription Services, Inc., A Wiley Company
简介
Objective
Axonal degeneration is a main contributor to disability in progressive neurodegenerative diseases in which oxidative stress is often identified as a pathogenic factor. We aim to demonstrate that antioxidants are able to improve axonal degeneration and locomotor deficits in a mouse model of X‐adrenoleukodystrophy (X‐ALD).
Methods
X‐ALD is a lethal disease caused by loss of function of the ABCD1 peroxisomal transporter of very long chain fatty acids (VLCFA). The mouse model for X‐ALD exhibits a late onset neurological phenotype with locomotor disability and axonal degeneration in spinal cord resembling the most common phenotype of the disease, adrenomyeloneuropathy (X‐AMN). Recently, we identified oxidative damage as an early event in life, and the excess of VLCFA as a generator of radical oxygen species (ROS) and oxidative damage to proteins in X‐ALD.
Results
Here, we prove the …
引用总数
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