Structure and mechanism of human ABC transporters

A Alam, KP Locher - Annual Review of Biophysics, 2023 - annualreviews.org
ABC transporters are essential for cellular physiology. Humans have 48 ABC genes
organized into seven distinct families. Of these genes, 44 (in five distinct families) encode for …

Structural and mechanistic principles of ABC transporters

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 …

[HTML][HTML] Carnitine transport and fatty acid oxidation

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 …

[HTML][HTML] Human disorders of peroxisome metabolism and biogenesis

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: a nexus for lipid metabolism and cellular signaling

IJ Lodhi, CF Semenkovich - Cell metabolism, 2014 - cell.com
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 …

X-linked adrenoleukodystrophy (X-ALD): clinical presentation and guidelines for diagnosis, follow-up and management

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 …

[HTML][HTML] Plant ABC transporters

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 …

The axon-myelin unit in development and degenerative disease

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 …

Adrenoleukodystrophy–neuroendocrine pathogenesis and redefinition of natural history

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 …

[HTML][HTML] Metabolism of very long-chain fatty acids: genes and pathophysiology

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 …