C9orf72-ALS human iPSC microglia are pro-inflammatory and toxic to co-cultured motor neurons via MMP9

• Published in: Nature communications Vol. 14; no. 1; p. 5898
• Main Authors: Vahsen, Björn F., Nalluru, Sumedha, Morgan, Georgia R., Farrimond, Lucy, Carroll, Emily, Xu, Yinyan, Cramb, Kaitlyn M. L., Amein, Benazir, Scaber, Jakub, Katsikoudi, Antigoni, Candalija, Ana, Carcolé, Mireia, Dafinca, Ruxandra, Isaacs, Adrian M., Wade-Martins, Richard, Gray, Elizabeth, Turner, Martin R., Cowley, Sally A., Talbot, Kevin
• Format: Journal Article
• Language: English
• Published: London Nature Publishing Group UK 22.09.2023; Nature Publishing Group; Nature Portfolio
• Subjects: 13/100; 13/106; 14/19; 38/91; 631/378/1689/1285; 631/378/2596/1953; 631/532/2064/2158; Amyotrophic lateral sclerosis; Amyotrophic Lateral Sclerosis - genetics; C9orf72 Protein - genetics; Cell activation; Cell culture; Chemokines; Coculture Techniques; Gelatinase B; Gene sequencing; Genetic diversity; Genetic variance; Humanities and Social Sciences; Humans; Immune system; Induced Pluripotent Stem Cells; Inflammation; Lipopolysaccharides; Matrix metalloproteinase; Matrix Metalloproteinase 9 - genetics; Matrix metalloproteinases; Metalloproteinase; Microglia; Motor Neurons; multidisciplinary; Mutants; Neurodegenerative Diseases; Neurons; Pathophysiology; Priming; Regulatory sequences; Science; Science (multidisciplinary); Therapeutic targets

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive motor neuron loss, with additional pathophysiological involvement of non-neuronal cells such as microglia. The commonest ALS-associated genetic variant is a hexanucleotide repeat expansion (HRE) mutation...