New Insights into the Mechanism of ALS

The damaging effects of toxic proteins created in one inherited form of amyotrophic lateral sclerosis (ALS) (Lou Gehrig’’s disease) are mediated by a protein called SPOP (speckle-type POZ protein) (image at left), according to a study published online on October 5, 2021 in PNAS. The article is titled “The Nuclear Ubiquitin Ligase Adaptor SPOP Is a Conserved Regulator of C9orf72 Dipeptide Toxicity,” and is authored by collaborators at Northwestern Feiberg School of Medicine and the University of Pittsburgh Medical Center.  Reducing the abundance of SPOP or inhibiting its activity with a small molecule could protect neurons against protein toxicity, according to Robert Kalb, MD, Director of the Les Turner ALS Center at the Northwestern Medicine Feinberg School of Medicine, the Joan and Paul Rubschlager Professor, and co-senior author of the study.
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