Essentiality of copper in humans

R Uauy, M Olivares, M Gonzalez - The American journal of clinical nutrition, 1998 - Elsevier
The biochemical basis for the essentiality of copper, the adequacy of the dietary copper
supply, factors that condition deficiency, and the special conditions of copper nutriture in …

Apoptosis in the nervous system

J Yuan, BA Yankner - Nature, 2000 - nature.com
Neuronal apoptosis sculpts the developing brain and has a potentially important role in
neurodegenerative diseases. The principal molecular components of the apoptosis …

The structural biochemistry of the superoxide dismutases

JJP Perry, DS Shin, ED Getzoff, JA Tainer - Biochimica et Biophysica Acta …, 2010 - Elsevier
The discovery of superoxide dismutases (SODs), which convert superoxide radicals to
molecular oxygen and hydrogen peroxide, has been termed the most important discovery of …

Molecular biology of amyotrophic lateral sclerosis: insights from genetics

P Pasinelli, RH Brown - Nature Reviews Neuroscience, 2006 - nature.com
Amyotrophic lateral sclerosis (ALS) is a paralytic disorder caused by motor neuron
degeneration. Mutations in more than 50 human genes cause diverse types of motor neuron …

Nearly 30 years of animal models to study amyotrophic lateral sclerosis: a historical overview and future perspectives

T Bonifacino, RA Zerbo, M Balbi, C Torazza… - International journal of …, 2021 - mdpi.com
Amyotrophic lateral sclerosis (ALS) is a fatal, multigenic, multifactorial, and non-cell
autonomous neurodegenerative disease characterized by upper and lower motor neuron …

The role of copper in neurodegenerative disease

DJ Waggoner, TB Bartnikas, JD Gitlin - Neurobiology of disease, 1999 - Elsevier
Copper is an essential trace metal which plays a fundamental role in the biochemistry of the
human nervous system. Menkes disease and Wilson disease are inherited disorders of …

Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue

S Zeitlin, JP Liu, DL Chapman, VE Papaioannou… - Nature …, 1995 - nature.com
The expansion of GAG triplet repeats in the translated region of the human HD gene,
encoding a protein (huntingtin) of unknown function, is a dominant mutation leading to …

Copper-zinc superoxide dismutase and amyotrophic lateral sclerosis

JS Valentine, PA Doucette, S Zittin Potter - Annu. Rev. Biochem., 2005 - annualreviews.org
▪ Abstract Copper-zinc superoxide dismutase (CuZnSOD, SOD1 protein) is an abundant
copper-and zinc-containing protein that is present in the cytosol, nucleus, peroxisomes, and …

ALS pathogenesis and therapeutic approaches: the role of mesenchymal stem cells and extracellular vesicles

R Bonafede, R Mariotti - Frontiers in cellular neuroscience, 2017 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by
progressive muscle paralysis determined by the degeneration of motoneurons in the motor …

Zinc metabolism in the brain: relevance to human neurodegenerative disorders

MP Cuajungco, GJ Lees - Neurobiology of disease, 1997 - Elsevier
Zinc is an important trace element in biology. An important pool of zinc in the brain is the one
present in synaptic vesicles in a subgroup of glutamatergic neurons. In this form it can be …