Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1

…, KL Anderson, SD Anderson, E Ohama, AG Reaume… - Science, 1998 - science.org
Analysis of transgenic mice expressing familial amyotrophic lateral sclerosis (ALS)–linked
mutations in the enzyme superoxide dismutase (SOD1) have shown that motor neuron death …

Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury

AG Reaume, JL Elliott, EK Hoffman, NW Kowall… - Nature …, 1996 - nature.com
The discovery that some cases of familial amyotrophic lateral sclerosis (FALS) are associated
with mutations in the gene encoding Cu/Zn superoxide dismutase (SOD1) has focused …

Cardiac malformation in neonatal mice lacking connexin43

AG Reaume, PA de Sousa, S Kulkarni, BL Langille… - Science, 1995 - science.org
Gap junctions are made up of connexin proteins, which comprise a multigene family in
mammals. Targeted mutagenesis of connexin43 (Cx43), one of the most prevalent connexin …

Notch1 is required for the coordinate segmentation of somites

RA Conlon, AG Reaume, J Rossant - Development, 1995 - journals.biologists.com
Members of the Notch family of transmembrane receptors mediate a number of developmental
decisions in invertebrates. In order to study Notch function in a vertebrate organism, we …

Reduction of CuZn-superoxide dismutase activity exacerbates neuronal cell injury and edema formation after transient focal cerebral ischemia

T Kondo, AG Reaume, TT Huang… - Journal of …, 1997 - Soc Neuroscience
Apoptotic neuronal cell death has recently been associated with the development of
infarction after cerebral ischemia. In a variety of studies, CuZn-superoxide dismutase (CuZn-SOD) …

Targeted deletion of the cytosolic Cu/Zn-superoxide dismutase gene (Sod1) increases susceptibility to noise-induced hearing loss

…, DL Ding, DG Flood, AG Reaume… - Audiology and …, 1999 - karger.com
Reactive oxygen species (ROS) such as superoxide, peroxide and hydroxyl radicals are
generated during normal cellular metabolism and are increased in acute injury and in many …

[HTML][HTML] Modulation of secreted β-amyloid precursor protein and amyloid β-peptide in brain by cholesterol

…, SP Trusko, MJ Savage, AG Reaume… - Journal of Biological …, 1998 - ASBMB
The effects of dietary cholesterol on brain amyloid precursor protein (APP) processing were
examined using an APP gene-targeted mouse, genetically humanized in the amyloid β-…

Turnover of amyloid β-protein in mouse brain and acute reduction of its level by phorbol ester

…, LR Pinsker, S Mistretta, AG Reaume… - Journal of …, 1998 - Soc Neuroscience
Fibrillar amyloid deposits are defining pathological lesions in Alzheimer’s disease brain and
are thought to mediate neuronal death. Amyloid is composed primarily of a 39–42 amino …

Mice lacking cytosolic copper/zinc superoxide dismutase display a distinctive motor axonopathy

JM Shefner, AG Reaume, DG Flood, RW Scott… - Neurology, 1999 - AAN Enterprises
Objective: To characterize the motor neuron dysfunction in two models by performing
physiologic and morphometric studies. Background: Mutations in the gene encoding cytosolic …

Age-related cochlear hair cell loss is enhanced in mice lacking copper/zinc superoxide dismutase

SL McFadden, D Ding, AG Reaume, DG Flood… - Neurobiology of …, 1999 - Elsevier
Age-related hearing loss in humans and many strains of mice is associated with a base-to-apex
gradient of cochlear hair cell loss. To determine if copper/zinc superoxide dismutase (Cu/…