Mechanisms of mutant SOD1 induced mitochondrial toxicity in amyotrophic lateral sclerosis

Recent progress in the genetics of motor neuron disease

(2014) Josef Finsterer et al.   European Journal of Medical Genetics

Mia40 and MINOS act in parallel with Ccs1 in the biogenesis of mitochondrial Sod1

(2013) Aksana Varabyova et al.   FEBS Journal

Distinctive features of the D101N and D101G variants of superoxide dismutase 1; two mutations that produce rapidly progressing motor neuron disease

(2013) Jacob Ayers et al.   JOURNAL OF NEUROCHEMISTRY

ALS and neuromuscular disease: in search of the Holy Grail

(2013) Matthew C Kiernan   LANCET NEUROLOGY

Dysregulation of intracellular copper homeostasis is common to transgenic mice expressing human mutant superoxide dismutase-1s regardless of their copper-binding abilities

(2013) Eiichi Tokuda et al.   NEUROBIOLOGY OF DISEASE

Wild-type Cu/Zn superoxide dismutase stabilizes mutant variants by heterodimerization

(2013) Anna Weichert et al.   NEUROBIOLOGY OF DISEASE

Mutant superoxide dismutase-1 indistinguishable from wild-type causes ALS

(2012) Matthis Synofzik et al.   HUMAN MOLECULAR GENETICS

Expression of wild-type human superoxide dismutase-1 in mice causes amyotrophic lateral sclerosis

(2012) K. S. Graffmo et al.   HUMAN MOLECULAR GENETICS

Composition of Soluble Misfolded Superoxide Dismutase-1 in Murine Models of Amyotrophic Lateral Sclerosis

(2012) Per Zetterström et al.   NEUROMOLECULAR MEDICINE

Human superoxide dismutase 1 (hSOD1) maturation through interaction with human copper chaperone for SOD1 (hCCS)

(2012) L. Banci et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Localization of a toxic form of superoxide dismutase 1 protein to pathologically affected tissues in familial ALS

(2012) T. E. Brotherton et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

VAPB interacts with the mitochondrial protein PTPIP51 to regulate calcium homeostasis

(2011) Kurt J. De Vos et al.   HUMAN MOLECULAR GENETICS

Mitochondrial Cu,Zn-Superoxide Dismutase Mediates Pulmonary Fibrosis by Augmenting H2O2Generation

(2011) Chao He et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Diacetylbis(N(4)-methylthiosemicarbazonato) Copper(II) (CuII(atsm)) Protects against Peroxynitrite-induced Nitrosative Damage and Prolongs Survival in Amyotrophic Lateral Sclerosis Mouse Model

(2011) Cynthia P. W. Soon et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mia40-dependent oxidation of cysteines in domain I of Ccs1 controls its distribution between mitochondria and the cytosol

(2011) Christine Klöppel et al.   MOLECULAR BIOLOGY OF THE CELL

Mitochondrial Ccs1 contains a structural disulfide bond crucial for the import of this unconventional substrate by the disulfide relay system

(2011) Dominik P. Groß et al.   MOLECULAR BIOLOGY OF THE CELL

Respiration and ROS production in brain and spinal cord mitochondria of transgenic rats with mutant G93a Cu/Zn-superoxide dismutase gene

(2011) Alexander Panov et al.   NEUROBIOLOGY OF DISEASE

Mutant SOD1 forms ion channel: Implications for ALS pathophysiology

(2011) Michael J. Allen et al.   NEUROBIOLOGY OF DISEASE

Oxidation and Reduction of Cysteines in the Intermembrane Space of Mitochondria: Multiple Facets of Redox Control

(2010) Johannes M. Herrmann et al.   ANTIOXIDANTS & REDOX SIGNALING

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

Copper depletion increases the mitochondrial-associated SOD1 in neuronal cells

(2010) Mario Arciello et al.   BIOMETALS

SOD1 targeted to the mitochondrial intermembrane space prevents motor neuropathy in the Sod1 knockout mouse

(2010) Lindsey R. Fischer et al.   BRAIN

Glutaredoxin 2 prevents aggregation of mutant SOD1 in mitochondria and abolishes its toxicity

(2010) Alberto Ferri et al.   HUMAN MOLECULAR GENETICS

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

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

Mutant SOD1 in neuronal mitochondria causes toxicity and mitochondrial dynamics abnormalities

(2009) J. Magrane et al.   HUMAN MOLECULAR GENETICS

Variation in aggregation propensities among ALS-associated variants of SOD1: Correlation to human disease

(2009) Mercedes Prudencio et al.   HUMAN MOLECULAR GENETICS

Wild-type SOD1 overexpression accelerates disease onset of a G85R SOD1 mouse

(2009) L. Wang et al.   HUMAN MOLECULAR GENETICS

Activation of Cu,Zn-Superoxide Dismutase in the Absence of Oxygen and the Copper Chaperone CCS

(2009) Jeffry M. Leitch et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Hypermetabolism in ALS patients: an early and persistent phenomenon

(2009) C. Bouteloup et al.   JOURNAL OF NEUROLOGY

Wild-type Cu/Zn superoxide dismutase (SOD1) does not facilitate, but impedes the formation of protein aggregates of amyotrophic lateral sclerosis causing mutant SOD1

(2009) Heidrun Witan et al.   NEUROBIOLOGY OF DISEASE

Role of mutant SOD1 disulfide oxidation and aggregation in the pathogenesis of familial ALS

(2009) C. M. Karch et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Ammonium tetrathiomolybdate delays onset, prolongs survival, and slows progression of disease in a mouse model for amyotrophic lateral sclerosis

(2008) Eiichi Tokuda et al.   EXPERIMENTAL NEUROLOGY

Heterodimer formation of wild-type and amyotrophic lateral sclerosis-causing mutant Cu/Zn-superoxide dismutase induces toxicity independent of protein aggregation

(2008) Heidrun Witan et al.   HUMAN MOLECULAR GENETICS

Different regulation of wild-type and mutant Cu,Zn superoxide dismutase localization in mammalian mitochondria

(2008) Hibiki Kawamata et al.   HUMAN MOLECULAR GENETICS

Deleterious Role of Superoxide Dismutase in the Mitochondrial Intermembrane Space

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

The Disulfide Relay System of Mitochondria Is Required for the Biogenesis of Mitochondrial Ccs1 and Sod1

(2008) Silvia Reddehase et al.   JOURNAL OF MOLECULAR BIOLOGY

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

Mutant SOD1 from spinal cord of G93A rats is destabilized and binds to inner mitochondrial membrane

(2008) Toni Ahtoniemi et al.   NEUROBIOLOGY OF DISEASE

Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria

(2008) C. Vande Velde et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA