Biochemical properties and in vivo effects of the SOD1 zinc-binding site mutant (H80G)

The Disulfide Relay of the Intermembrane Space of Mitochondria: An Oxygen-Sensing System?

(2010) Karl Bihlmaier et al.   Annals of the New York Academy of Sciences

Disrupted Zinc-Binding Sites in Structures of Pathogenic SOD1 Variants D124V and H80R

(2010) Sai V. Seetharaman et al.   BIOCHEMISTRY

Cu,Zn-Superoxide Dismutase Increases Toxicity of Mutant and Zinc-deficient Superoxide Dismutase by Enhancing Protein Stability

(2010) Mary Anne Sahawneh et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Exposure of Hydrophobic Surfaces Initiates Aggregation of Diverse ALS-Causing Superoxide Dismutase-1 Mutants

(2010) Christian Münch et al.   JOURNAL OF MOLECULAR BIOLOGY

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 ALS-Associated Mutation G93A in Human Copper-Zinc Superoxide Dismutase Selectively Destabilizes the Remote Metal Binding Region

(2009) Anna Katrine Museth et al.   BIOCHEMISTRY

Immature Copper-Zinc Superoxide Dismutase and Familial Amyotrophic Lateral Sclerosis

(2009) Sai V. Seetharaman et al.   EXPERIMENTAL BIOLOGY AND MEDICINE

Metal Deficiency Increases Aberrant Hydrophobicity of Mutant Superoxide Dismutases That Cause Amyotrophic Lateral Sclerosis

(2009) Ashutosh Tiwari et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Redox susceptibility of SOD1 mutants is associated with the differential response to CCS over-expression in vivo

(2009) Marjatta Son 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

The Erv1–Mia40 disulfide relay system in the intermembrane space of mitochondria

(2008) Kai Hell   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Biological effects of CCS in the absence of SOD1 enzyme activation: implications for disease in a mouse model for ALS

(2008) Jody B. Proescher 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

Isolated CytochromecOxidase Deficiency in G93A SOD1 Mice Overexpressing CCS Protein

(2008) Marjatta Son 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