Structural basis of substrate recognition and translocation by human ABCD1

ZP Chen, D Xu, L Wang, CZ Zhou, WT Hou, Y Chen - bioRxiv, 2021 - biorxiv.org
Human ATP-binding cassette (ABC) transporter ABCD1 transports CoA esters of saturated/
monounsaturated very long chain fatty acid from cytosol to the peroxisome for β-oxidation …

[PDF][PDF] BBA-Molecular and Cell Biology of Lipids

Q Raas, FE Saih, C Gondcaille, D Trompier, Y Hamon… - wiki.uio.no
ABSTRACT X-linked adrenoleukodystrophy (X-ALD), the most frequent peroxisomal
disorder, is associated with mutation in the ABCD1 gene which encodes a peroxisomal ATP …

[PDF][PDF] ER-to-Peroxisome tethering and Membrane Rearrangement during Peroxisome Maintenance

M Johannes-Paul - core.ac.uk
The work I am presenting herein is a mere compilation of what I have contributed to during
the last years. Although the breakthroughs, the highlights have always been the driving force …

Function of Peroxisome in Mammal and Analysis of the Fatty Acid Oxidation System by Photoaffinity Labeling

T Imanaka, K Kawaguchi, M Morita - Photoaffinity Labeling for Structural …, 2017 - Springer
Peroxisomes play an essential role in a number of important metabolic pathways including
oxidation of fatty acids, and synthesis of ether phospholipids and bile acids. Long, very long …

AC elegans model for X-linked adrenoleukodystrophy: roles of pmp-4 fatty acid transporter in the nervous system

SK Guha - 2015 - tdx.cat
X-linked adrenoleukodystrophy (X-ALD) is an inherited neurodegenerative disorder. The
genetic bases for all its different phenotypic variants are mutation in the gene encoding the …

Physiological Functions of Cl-/HCO3-Exchangers and Na+/HCO3-Cotransporters in Heart

K Vairamani - 2015 - rave.ohiolink.edu
The function of bicarbonate transporters has been extensively studied in epithelial tissues,
but their function in heart is not well understood. Cardiac tissue expresses an abundance of …

[PDF][PDF] PATHOGENESIS OF HYPERCHOLESTEROLEMIA IN IL-13-INDUCED RAT MODEL OF MINIMAL CHANGE NEPHROTIC SYNDROME

LOWD LAURETTA - 2015 - core.ac.uk
One of the major complications of minimal change nephrotic syndrome (MCNS) is
hypercholesterolemia. In non-MCNS experimental rat models of NS, hypercholesterolemia …

[PDF][PDF] Author's Accepted Manuscript

S Fourcade, I Ferrer, A Pujol - 2015 - cyberleninka.org
Oxidative stress, mitochondrial and proteostasis malfunction in adrenoleukodystrophy_ A
paradigm for axonal degeneration Page 1 Author's Accepted Manuscript Oxidative stress …

[PDF][PDF] Abcd2 Is a Strong Modifier of the Metabolic Impairments in Peritoneal

Z Muneer, C Wiesinger, T Voigtländer, HB Werner… - 2014 - meduniwien.ac.at
The inherited peroxisomal disorder X-linked adrenoleukodystrophy (X-ALD), associated with
neurodegeneration and inflammatory cerebral demyelination, is caused by mutations in the …

Beyond Peroxisome: ABCD2 Modifies PPARα Signaling and Identifies a Subclass of Peroxisomes in Mouse Adipose Tissue

X Liu - 2014 - uknowledge.uky.edu
Abstract ABCD2 (D2) has been proposed as a peroxisomal long-chain acyl-CoA transporter
that is essential for very long chain fatty acid metabolism. In the livers of mice, D2 is highly …