Mitochondrial pathobiology in ALS

Glycine Receptor Channels in Spinal Motoneurons Are Abnormal in a Transgenic Mouse Model of Amyotrophic Lateral Sclerosis

(2011) Q. Chang et al.   JOURNAL OF NEUROSCIENCE

Skeletal muscle-restricted expression of human SOD1 causes motor neuron degeneration in transgenic mice

(2010) Margaret Wong et al.   HUMAN MOLECULAR GENETICS

Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS

(2010) Andrew C. Elden et al.   NATURE

Mutations of optineurin in amyotrophic lateral sclerosis

(2010) Hirofumi Maruyama et al.   NATURE

Current and emerging treatments for amyotrophic lateral sclerosis

(2010) Zoccolella   Neuropsychiatric Disease and Treatment

Deleterious Variants of FIG4, a Phosphoinositide Phosphatase, in Patients with ALS

(2009) Clement Y. Chow et al.   AMERICAN JOURNAL OF HUMAN GENETICS

A Role for Copper in the Toxicity of Zinc-Deficient Superoxide Dismutase to Motor Neurons in Amyotrophic Lateral Sclerosis

(2009) Kari A. Trumbull et al.   ANTIOXIDANTS & REDOX SIGNALING

The mitochondrial permeability transition pore: A molecular target for amyotrophic lateral sclerosis therapy

(2009) Lee J. Martin   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Inducible nitric oxide synthase is present in motor neuron mitochondria and Schwann cells and contributes to disease mechanisms in ALS mice

(2009) Kevin Chen et al.   Brain Structure & Function

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

Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6

(2009) C. Vance et al.   SCIENCE

Glycinergic Innervation of Motoneurons Is Deficient in Amyotrophic Lateral Sclerosis Mice

(2008) Qing Chang et al.   AMERICAN JOURNAL OF PATHOLOGY

Recent progress in elucidating the molecular mechanism of the mitochondrial permeability transition pore

(2008) Anna W.C. Leung et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Ro5-4864 promotes neonatal motor neuron survival and nerve regeneration in adult rats

(2008) Charles Mills et al.   EUROPEAN JOURNAL OF NEUROSCIENCE

Calcium-induced generation of reactive oxygen species in brain mitochondria is mediated by permeability transition

(2008) Magnus J. Hansson et al.   FREE RADICAL BIOLOGY AND MEDICINE

Deleterious Role of Superoxide Dismutase in the Mitochondrial Intermembrane Space

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

Expression of mutant SOD1G93Ain astrocytes induces functional deficits in motoneuron mitochondria

(2008) Lynsey G. Bilsland et al.   JOURNAL OF NEUROCHEMISTRY

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

TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis

(2008) Edor Kabashi et al.   NATURE GENETICS

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

(2008) B TURNER et al.   PROGRESS IN NEUROBIOLOGY