Regulation of cellular iron metabolism

J Wang, K Pantopoulos - Biochemical Journal, 2011 - portlandpress.com
Iron is an essential but potentially hazardous biometal. Mammalian cells require sufficient
amounts of iron to satisfy metabolic needs or to accomplish specialized functions. Iron is …

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 …

Mitochondrial disease

AHV Schapira - The Lancet, 2006 - thelancet.com
Defects of mitochondrial metabolism cause a wide range of human diseases that include
examples from all medical subspecialties. This review updates the topic of mitochondrial …

Mitochondria take center stage in aging and neurodegeneration

MF Beal - Annals of Neurology: Official Journal of the American …, 2005 - Wiley Online Library
A critical role of mitochondrial dysfunction and oxidative damage has been hypothesized in
both aging and neurodegenerative diseases. Much of the evidence has been correlative, but …

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 …

Iron behaving badly: inappropriate iron chelation as a major contributor to the aetiology of vascular and other progressive inflammatory and degenerative diseases

DB Kell - BMC medical genomics, 2009 - Springer
Background The production of peroxide and superoxide is an inevitable consequence of
aerobic metabolism, and while these particular'reactive oxygen species'(ROSs) can exhibit a …

Maturation of iron-sulfur proteins in eukaryotes: mechanisms, connected processes, and diseases

R Lill, U Mühlenhoff - Annu. Rev. Biochem., 2008 - annualreviews.org
Iron-sulfur (Fe/S) proteins are involved in a wide variety of cellular processes such as
enzymatic reactions, respiration, cofactor biosynthesis, ribosome biogenesis, regulation of …

The proteasomal system

T Jung, B Catalgol, T Grune - Molecular aspects of medicine, 2009 - Elsevier
Rising interest in the mechanism and function of the proteasomes and the ubiquitin system
revealed that it is hard to find any aspect of the cellular metabolic network that is not directly …

Mitochondrial iron–sulfur protein biogenesis and human disease

O Stehling, C Wilbrecht, R Lill - Biochimie, 2014 - Elsevier
Work during the past 14 years has shown that mitochondria are the primary site for the
biosynthesis of iron–sulfur (Fe/S) clusters. In fact, it is this process that renders mitochondria …