TY  - JOUR
AU  - Coppa, Andrea
AU  - Guha, Sanjib
AU  - Fourcade, Stéphane
AU  - Parameswaran, Janani
AU  - Ruiz, Montserrat
AU  - Moser, Ann B
AU  - Schlüter, Agatha
AU  - Murphy, Michael P
AU  - Lizcano, Jose Miguel
AU  - Miranda-Vizuete, Antonio
AU  - Dalfó, Esther
AU  - Pujol, Aurora
PY  - 2020
DO  - 10.1016/j.freeradbiomed.2020.01.177
UR  - http://hdl.handle.net/10668/15041
T2  - Free radical biology & medicine
AB  - Adrenoleukodystrophy is a neurometabolic disorder caused by a defective peroxisomal ABCD1 transporter of very long-chain fatty acids (VLCFAs). Its pathogenesis is incompletely understood. Here we characterize a nematode model of X-ALD with loss of the...
LA  - en
KW  - Axonal degeneration
KW  - Hypodermis
KW  - Lipid droplets
KW  - Mitochondria redox imbalance
KW  - Peroxisomes
KW  - X-linked adrenoleukodystrophy
KW  - ATP Binding Cassette Transporter, Subfamily D, Member 1
KW  - ATP-Binding Cassette Transporters
KW  - Adrenoleukodystrophy
KW  - Animals
KW  - Caenorhabditis elegans
KW  - Fatty Acids
KW  - Mice
KW  - Mice, Knockout
KW  - Subcutaneous Tissue
TI  - The peroxisomal fatty acid transporter ABCD1/PMP-4 is required in the C. elegans hypodermis for axonal maintenance: A worm model for adrenoleukodystrophy.
TY  - research article
VL  - 152
ER  -