Myelination of the nervous system: mechanisms and functions

KA Nave, HB Werner - Annual review of cell and developmental …, 2014 - annualreviews.org
Myelination of axons in the nervous system of vertebrates enables fast, saltatory impulse
propagation, one of the best-understood concepts in neurophysiology. However, it took a …

X‐linked adrenoleukodystrophy: pathology, pathophysiology, diagnostic testing, newborn screening and therapies

BR Turk, C Theda, A Fatemi… - International Journal of …, 2020 - Wiley Online Library
Adrenoleukodystrophy (ALD) is a rare X‐linked disease caused by a mutation of the
peroxisomal ABCD1 gene. This review summarizes our current understanding of the …

[HTML][HTML] Redox interplay between mitochondria and peroxisomes

C Lismont, M Nordgren, PP Van Veldhoven… - Frontiers in cell and …, 2015 - frontiersin.org
Reduction-oxidation or “redox” reactions are an integral part of a broad range of cellular
processes such as gene expression, energy metabolism, protein import and folding, and …

[HTML][HTML] Peroxisomes in brain development and function

J Berger, F Dorninger, S Forss-Petter… - Biochimica Et Biophysica …, 2016 - Elsevier
Peroxisomes contain numerous enzymatic activities that are important for mammalian
physiology. Patients lacking either all peroxisomal functions or a single enzyme or …

The advanced lipoxidation end-product malondialdehyde-lysine in aging and longevity

M Jové, N Mota-Martorell, I Pradas, M Martín-Gari… - Antioxidants, 2020 - mdpi.com
The nonenzymatic adduction of malondialdehyde (MDA) to the protein amino groups leads
to the formation of malondialdehyde-lysine (MDALys). The degree of unsaturation of …

Pharmacological advances in mitochondrial therapy

A Singh, D Faccenda, M Campanella - EBioMedicine, 2021 - thelancet.com
Mitochondria play a vital role in cellular metabolism and are central mediator of intracellular
signalling, cell differentiation, morphogenesis and demise. An increasingly higher number of …

Adulthood leukodystrophies

W Köhler, J Curiel, A Vanderver - Nature Reviews Neurology, 2018 - nature.com
The leukodystrophies are a group of inherited white matter disorders with a heterogeneous
genetic background, considerable phenotypic variability and disease onset at all ages. This …

Enhanced axonal response of mitochondria to demyelination offers neuroprotection: implications for multiple sclerosis

S Licht-Mayer, GR Campbell, M Canizares… - Acta …, 2020 - Springer
Axonal loss is the key pathological substrate of neurological disability in demyelinating
disorders, including multiple sclerosis (MS). However, the consequences of demyelination …

The changing face of adrenoleukodystrophy

J Zhu, F Eichler, A Biffi, CN Duncan… - Endocrine …, 2020 - academic.oup.com
Adrenoleukodystrophy (ALD) is a rare X-linked disorder of peroxisomal oxidation due to
mutations in ABCD1. It is a progressive condition with a variable clinical spectrum that …

Peroxisomal ABC transporters: an update

A Tawbeh, C Gondcaille, D Trompier… - International Journal of …, 2021 - mdpi.com
ATP-binding cassette (ABC) transporters constitute one of the largest superfamilies of
conserved proteins from bacteria to mammals. In humans, three members of this family are …