The role of oxidative stress and hypoxia in pancreatic beta-cell dysfunction in diabetes mellitus

PA Gerber, GA Rutter - Antioxidants & redox signaling, 2017 - liebertpub.com
Significance: Metabolic syndrome is a frequent precursor of type 2 diabetes mellitus (T2D), a
disease that currently affects∼ 8% of the adult population worldwide. Pancreatic beta-cell …

Iron, brain ageing and neurodegenerative disorders

L Zecca, MBH Youdim, P Riederer, JR Connor… - Nature Reviews …, 2004 - nature.com
There is increasing evidence that iron is involved in the mechanisms that underlie many
neurodegenerative diseases. Conditions such as neuroferritinopathy and Friedreich ataxia …

Functional spectrum and specificity of mitochondrial ferredoxins FDX1 and FDX2

V Schulz, S Basu, SA Freibert, H Webert, L Boss… - Nature chemical …, 2023 - nature.com
Ferredoxins comprise a large family of iron–sulfur (Fe–S) proteins that shuttle electrons in
diverse biological processes. Human mitochondria contain two isoforms of [2Fe-2S] …

[HTML][HTML] The role of mitochondria in cellular iron–sulfur protein biogenesis and iron metabolism

R Lill, B Hoffmann, S Molik, AJ Pierik… - … et Biophysica Acta (BBA …, 2012 - Elsevier
Mitochondria play a key role in iron metabolism in that they synthesize heme, assemble iron–
sulfur (Fe/S) proteins, and participate in cellular iron regulation. Here, we review the latter …

Trinucleotide repeat disorders

HT Orr, HY Zoghbi - Annu. Rev. Neurosci., 2007 - annualreviews.org
The discovery that expansion of unstable repeats can cause a variety of neurological
disorders has changed the landscape of disease-oriented research for several forms of …

[HTML][HTML] Reprint of: Iron/sulfur proteins biogenesis in prokaryotes: formation, regulation and diversity

B Roche, L Aussel, B Ezraty, P Mandin, B Py… - … et Biophysica Acta (BBA …, 2013 - Elsevier
Iron/sulfur centers are key cofactors of proteins intervening in multiple conserved cellular
processes, such as gene expression, DNA repair, RNA modification, central metabolism and …

Friedreich's ataxia: pathology, pathogenesis, and molecular genetics

AH Koeppen - Journal of the neurological sciences, 2011 - Elsevier
The pathogenic mutation in Friedreich's ataxia (FRDA) is a homozygous guanine-adenine-
adenine (GAA) trinucleotide repeat expansion on chromosome 9q13 that causes a …

Diseases of unstable repeat expansion: mechanisms and common principles

JR Gatchel, HY Zoghbi - Nature Reviews Genetics, 2005 - nature.com
The list of developmental and degenerative diseases that are caused by expansion of
unstable repeats continues to grow, and is now approaching 20 disorders. The pathogenic …

Intracellular iron transport and storage: from molecular mechanisms to health implications

EL Mackenzie, K Iwasaki, Y Tsuji - Antioxidants & redox signaling, 2008 - liebertpub.com
Maintenance of proper “labile iron” levels is a critical component in preserving homeostasis.
Iron is a vital element that is a constituent of a number of important macromolecules …

Structure and functional dynamics of the mitochondrial Fe/S cluster synthesis complex

MT Boniecki, SA Freibert, U Mühlenhoff, R Lill… - Nature …, 2017 - nature.com
Abstract Iron–sulfur (Fe/S) clusters are essential protein cofactors crucial for many cellular
functions including DNA maintenance, protein translation, and energy conversion. De novo …