C Thomas, R Tampé - Annual review of biochemistry, 2020 - annualreviews.org
ATP-binding cassette (ABC) transporters constitute one of the largest and most ancient protein superfamilies found in all living organisms. They function as molecular machines by …
N Longo, M Frigeni, M Pasquali - … et Biophysica Acta (BBA)-Molecular Cell …, 2016 - Elsevier
Carnitine is essential for the transfer of long-chain fatty acids across the inner mitochondrial membrane for subsequent β-oxidation. It can be synthesized by the body or assumed with …
HR Waterham, S Ferdinandusse… - Biochimica Et Biophysica …, 2016 - Elsevier
Peroxisomes are dynamic organelles that play an essential role in a variety of cellular catabolic and anabolic metabolic pathways, including fatty acid alpha-and beta-oxidation …
Peroxisomes are often dismissed as the cellular hoi polloi, relegated to cleaning up reactive oxygen chemical debris discarded by other organelles. However, their functions extend far …
M Engelen, S Kemp, M De Visser, BM van Geel… - Orphanet journal of rare …, 2012 - Springer
Abstract X-linked adrenoleukodystrophy (X-ALD) is the most common peroxisomal disorder. The disease is caused by mutations in the ABCD1 gene that encodes the peroxisomal …
J Kang, J Park, H Choi, B Burla… - The Arabidopsis book …, 2011 - ncbi.nlm.nih.gov
ABC transporters constitute one of the largest protein families found in all living organisms. ABC transporters are driven by ATP hydrolysis and can act as exporters as well as …
RM Stassart, W Möbius, KA Nave… - Frontiers in …, 2018 - frontiersin.org
Axons are electrically excitable, cable-like neuronal processes that relay information between neurons within the nervous system and between neurons and peripheral target …
S Kemp, IC Huffnagel, GE Linthorst… - Nature Reviews …, 2016 - nature.com
X-Linked adrenoleukodystrophy (ALD) is a peroxisomal metabolic disorder with a highly complex clinical presentation. ALD is caused by mutations in the ABCD1 gene, which leads …
T Sassa, A Kihara - Biomolecules & therapeutics, 2014 - ncbi.nlm.nih.gov
Fatty acids (FAs) are highly diverse in terms of carbon (C) chain-length and number of double bonds. FAs with C> 20 are called very long-chain fatty acids (VLCFAs). VLCFAs are …