Amyotrophic lateral Ssclerosis and structural defects in cu, Zn superoxide dismutase

…, WY Hung, ED Getzoff, P Hu, B Herzfeldt, RP Roos… - Science, 1993 - science.org
Single-site mutants in the Cu,Zn superoxide dismutase (SOD) gene (SOD1) occur in
patients with the fatal neurodegenerative disorder familial amyotrophic lateral sclerosis (FALS). …

[PDF][PDF] TIA1 mutations in amyotrophic lateral sclerosis and frontotemporal dementia promote phase separation and alter stress granule dynamics

…, J Keith, SA Zivkovic, V Hirsch-Reinshagen, RP Roos… - Neuron, 2017 - cell.com
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are age-related
neurodegenerative disorders with shared genetic etiologies and overlapping clinical and …

Astrocyte loss of mutant SOD1 delays ALS disease onset and progression in G85R transgenic mice

L Wang, DH Gutmann, RP Roos - Human molecular genetics, 2011 - academic.oup.com
Approximately 10% of patients with amyotrophic lateral sclerosis (ALS) have familial ALS (FALS),
and 20% of FALS are caused by mutations of superoxide dismutase type 1 (MTSOD1). …

Patients with amyotrophic lateral sclerosis receiving long-term mechanical ventilation: advance care planning and outcomes

…, EA Oppenheimer, P Casey, PA Cazzolli, RP Roos… - Chest, 1996 - Elsevier
… Patients living at home rated their quality of life on a 10-point scale better than those in an
institution (7.2 vs 5.6; p=0.0052), and their yearly expenses were less ($136,560 vs $366,852; …

The unfolded protein response in familial amyotrophic lateral sclerosis

L Wang, B Popko, RP Roos - Human molecular genetics, 2011 - academic.oup.com
Mutant superoxide dismutase type 1 (MTSOD1) is thought to cause ∼20% of cases of familial
amyotrophic lateral sclerosis (FALS) because it misfolds and aggregates. Previous studies …

Guanabenz, which enhances the unfolded protein response, ameliorates mutant SOD1-induced amyotrophic lateral sclerosis

L Wang, B Popko, E Tixier, RP Roos - Neurobiology of disease, 2014 - Elsevier
Approximately 20% of familial amyotrophic lateral sclerosis (FALS) cases are caused by
mutant superoxide dismutase type 1 (mtSOD1). Although the mechanisms of mtSOD1-induced …

The effect of mutant SOD1 dismutase activity on non-cell autonomous degeneration in familial amyotrophic lateral sclerosis

L Wang, K Sharma, G Grisotti, RP Roos - Neurobiology of disease, 2009 - Elsevier
Mutant superoxide dismutase type 1 (MTSOD1), the most common known cause of familial
amyotrophic lateral sclerosis (FALS), is believed to cause FALS as a result of a toxicity of the …

Neuromuscular transmission in amyotrophic lateral sclerosis

…, EF Salazar‐Grueso, RP Roos - Muscle & Nerve …, 1993 - Wiley Online Library
The functional and structural characteristics of the neuromuscular junction were studied in
anconeus muscle biopsies of 10 patients with amyotrophic lateral sclerosis (ALS). Intracellular …

Amyotrophic lateral sclerosis and life-sustaining therapy: patients' desires for information, participation in decision making, and life-sustaining therapy

…, CB Stocking, JP Antel, J Beckwith, RP Roos… - Mayo Clinic …, 1991 - Elsevier
To identify the wishes of patients with amyotrophic lateral sclerosis (ALS) for information,
participation in decision making, and life-sustaining therapy and to determine whether these …

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

…, G Grisotti, H Zhai, T Siddique, RP Roos - Human molecular …, 2009 - academic.oup.com
Approximately 10% of amyotrophic lateral sclerosis (ALS) cases are familial (FALS), and ∼25%
of FALS cases are caused by mutations in Cu/Zn superoxide dismutase type 1 (SOD1). …