Both beneficial and neurotoxic forms of the mineral selenium were increased in people with SOD1-associated amyotrophic lateral sclerosis (ALS) after six months of treatment with Qalsody (tofersen), a report shows. The elevations, which have been linked to ALS previously, could reflect a change in the antioxidant status of…

The klotho protein may have brain-protecting effects in amyotrophic lateral sclerosis (ALS), according to a study in mice that may become a basis for developing new therapies. The study, “Klotho Is Neuroprotective in the Superoxide Dismutase (SOD1G93A) Mouse Model of ALS,” was published in the Journal…

SOD1 protein aggregates can spread in a prion-like way, rapidly causing amyotrophic lateral sclerosis (ALS) when injected into the spinal cord of mice carrying a human mutated SOD1 gene. The results, published in the Journal of Clinical Investigation, substantially advance insights into mechanisms in hereditary ALS, and…

By introducing a mutation in SOD1 — the protein believed to be responsible for a large part of amyotrophic lateral sclerosis (ALS) cases — researchers managed to prevent the protein from clumping in a way that kills neurons. The study, “A Phosphomimetic Mutation Stabilizes SOD1 and Rescues…

Clearance was given to open a Phase 1/2 trial evaluating APB-102, a potential gene therapy for amyotrophic lateral sclerosis (ALS) patients who carry mutations in the SOD1 gene, its developer Apic Bio announced. The trial is scheduled to begin later this year or early next year, following U.S. Food and Drug Administration…

Scientists at Umeå University have found that  superoxide dysmutase (SOD1), a protein that may cause amylotophic lateral sclerosis (ALS) spreads and clumps when injected into mice. The protein deposits also cause ALS-like symptoms. The report, titled “Two superoxide dismutase prion strains transmit amyotrophic lateral sclerosis-like disease” appeared May 3, 2016…

Severe DNA damage and other disease-associated hallmarks are absent in a mouse model of familial amyothrophic lateral sclerosis (fALS), a new study shows. The study, “DNA strand breaks and TDP-43 mislocation are absent in the murine hSOD1G93A model of amyotrophic lateral sclerosis in vivo and in vitro,” appeared in the journal…

A newly developed method can help characterize the types of misfolded proteins present in patients with amyotrophic lateral sclerosis (ALS), according to findings in a thesis. The recently published doctoral thesis, titled “Structural investigation of SOD1 aggregates in ALS: identification of prion strains using anti-peptide antibodies,” was written…

The ALS (amyotrophic lateral sclerosis) Association revealed that a team of researchers funded by the Association and led by Dr. Timothy Miller and Dr. Randall Bateman from Washington University in Saint Louis, recently discovered important insights into the ALS-related protein called copper (Cu)/zinc (Zn) superoxide dismutase (SOD1). ALS…

The former malaria therapy pyrimethamine reduces levels of an ALS-linked protein in the spinal fluid of patients whose disease stems from gene mutations, a Phase 1/2 clinical trial shows. The protein is SOD1. The mutations connected with amyotrophic lateral sclerosis are in the SOD1 gene that controls the protein. The…