«ALS is one of the most devastating human diseases and new therapies are urgently needed that can affect its underlying biology.»
Michael Salzmann, COO
Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease marked by the degeneration of motor neurons in the spinal cord and brain resulting in progressing paralysis and death. In absence of effective treatment, patients survive on average two to five years following their diagnosis. The worldwide population of ALS patients is estimated 450,000.
For the potential future treatment of ALS, Neurimmune developed antibodies against misfolded forms of superoxide dismutase 1 (SOD1), TAR DNA binding protein 43 (TDP43) and C9orf72 related peptides. Both TDP43 and C9orf72 are genetic causes of ALS and frontotemporal dementia.
Misfolded SOD1 accumulates in spinal cord and brain of patients with ALS, suggesting a role as a drug target. Neurimmune’s SOD1 lead antibody is highly effective at therapeutic doses in transgenic models of ALS: Chronic antibody treatment significantly reduces SOD1 pathology and rescues spinal cord motor neurons, resulting in less muscle atrophy, improved motor functions and increased survival times.
Neurimmune co-founded and co-financed the subsidiary AL-S Pharma AG to develop the SOD1 antibody AP-101 (NI005) to clinical proof-of-concept in collaboration with TVM Capital Life Science and Lilly's Chorus unit.
Neurimmune collaborates with Biogen on the C9orf72 program, and Biogen acquired Neurimmune's TDP43 antibodies.