Mitochondrial dysfunction in familial amyotrophic lateral sclerosis

G37R SOD1 mutant alters mitochondrial complex I activity, Ca2+ uptake and ATP production

(2011) Evelyne Coussee et al.   CELL CALCIUM

Repetitive nerve stimulation transiently opens the mitochondrial permeability transition pore in motor nerve terminals of symptomatic mutant SOD1 mice

(2011) Khanh T. Nguyen et al.   NEUROBIOLOGY OF DISEASE

Calcium dysregulation, mitochondrial pathology and protein aggregation in a culture model of amyotrophic lateral sclerosis: Mechanistic relationship and differential sensitivity to intervention

(2011) Miranda L. Tradewell et al.   NEUROBIOLOGY OF DISEASE

Review: The role of mitochondria in the pathogenesis of amyotrophic lateral sclerosis

(2011) L. M. Duffy et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

In yeast redistribution of Sod1 to the mitochondrial intermembrane space provides protection against respiration derived oxidative stress

(2010) Christine Klöppel et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Calcium dysregulation in amyotrophic lateral sclerosis

(2010) Julian Grosskreutz et al.   CELL CALCIUM

VDAC, a multi-functional mitochondrial protein regulating cell life and death

(2010) Varda Shoshan-Barmatz et al.   MOLECULAR ASPECTS OF MEDICINE

Misfolded Mutant SOD1 Directly Inhibits VDAC1 Conductance in a Mouse Model of Inherited ALS

(2010) Adrian Israelson et al.   NEURON

Axonal Mitochondrial Clusters Containing Mutant SOD1 in Transgenic Models of ALS

(2009) Jose R. Sotelo-Silveira et al.   ANTIOXIDANTS & REDOX SIGNALING

Oligomerization of Mutant SOD1 in Mitochondria of Motoneuronal Cells Drives Mitochondrial Damage and Cell Toxicity

(2009) Mauro Cozzolino et al.   ANTIOXIDANTS & REDOX SIGNALING

Impairment of mitochondrial calcium handling in a mtSOD1 cell culture model of motoneuron disease

(2009) Manoj Jaiswal et al.   BMC NEUROSCIENCE

Oxidative stress in ALS: Key role in motor neuron injury and therapeutic target

(2009) Siân C. Barber et al.   FREE RADICAL BIOLOGY AND MEDICINE

Voltage-dependent Anion Channel 1-based Peptides Interact with Bcl-2 to Prevent Antiapoptotic Activity

(2009) Nir Arbel et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The Ψmdepolarization that accompanies mitochondrial Ca2+uptake is greater in mutant SOD1 than in wild-type mouse motor terminals

(2009) Khanh T. Nguyen et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

How mitochondria produce reactive oxygen species

(2008) Michael P. Murphy   BIOCHEMICAL JOURNAL

Voltage dependent anion channels (VDACs): a brief introduction with a focus on the outer mitochondrial compartment’s roles together with hexokinase-2 in the “Warburg effect” in cancer

(2008) Peter L. Pedersen   JOURNAL OF BIOENERGETICS AND BIOMEMBRANES

Deleterious Role of Superoxide Dismutase in the Mitochondrial Intermembrane Space

(2008) Gundars Goldsteins et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Lysyl-tRNA Synthetase Is a Target for Mutant SOD1 Toxicity in Mitochondria

(2008) Hibiki Kawamata et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Isolated CytochromecOxidase Deficiency in G93A SOD1 Mice Overexpressing CCS Protein

(2008) Marjatta Son et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Cu/Zn Superoxide Dismutase Typical for Familial Amyotrophic Lateral Sclerosis Increases the Vulnerability of Mitochondria and Perturbs Ca2+ Homeostasis in SOD1G93A Mice

(2008) M. K. Jaiswal et al.   MOLECULAR PHARMACOLOGY

Low Ca2+ buffering in hypoglossal motoneurons of mutant SOD1 (G93A) mice

(2008) Friederike von Lewinski et al.   NEUROSCIENCE LETTERS

Transgenics, toxicity and therapeutics in rodent models of mutant SOD1-mediated familial ALS

(2008) B TURNER et al.   PROGRESS IN NEUROBIOLOGY