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Here, we demonstrate that the complement protein C1q is required for the refinement of sensory-motor circuits during normal development, as well as for synaptic dysfunction and elimination in spinal muscular atrophy (SMA). C1q tags vulnerable SMA synapses, which triggers activation of the classical complement pathway leading to microglia-mediated elimination. Pharmacological inhibition of C1q or depletion of microglia rescues the number and function of synapses, conferring significant behavioral benefit in SMA mice. Thus, the classical complement pathway plays critical roles in the refinement of developing motor circuits, while its aberrant activation contributes to motor neuron disease.","is_dataset_classified":null,"base_score":4.867534450455582,"endowment":4.867534450455582,"datacite_reuse_total":6,"file_count":0,"downloads":0,"views":0,"has_version_chain":false,"is_dataset":false,"is_oa":false,"pmid":"31801075","pmcid":"PMC6937140","openalex_id":"https://openalex.org/W2993235793","authors":[],"funders":[{"funder_name":"National Institutes of Health","grant_id":"R01-NS078375","title":null},{"funder_name":"U.S. Department of Defense","grant_id":"GR.10235006","title":null},{"funder_name":"National Institute on Alcohol Abuse and Alcoholism","grant_id":"R01-AA027079","title":null},{"funder_name":"NIAAA NIH HHS","grant_id":"R01 AA027079","title":null},{"funder_name":"NINDS NIH HHS","grant_id":"R01 NS078375","title":null},{"funder_name":"National Institutes of Health","grant_id":"1R01AA027079-01","title":"Mechanisms of synaptic loss by the classical complement pathway in motor circuit development and disease"},{"funder_name":"National Institutes of Health","grant_id":"1R01NS078375-01","title":"Mechanisms of Central Synaptic Dysfunction in SMA"},{"funder_name":"Spinal Muscular Atrophy Foundation","grant_id":"","title":null},{"funder_name":"National Institute of Neurological Disorders and Stroke","grant_id":"","title":null},{"funder_name":"Columbia University","grant_id":"","title":null},{"funder_name":"NIH Blueprint for Neuroscience 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and Muscular Disorders Research","Neuroinflammation and Neurodegeneration Mechanisms","Spinal Cord Injury Research","0301 basic medicine","0303 health sciences","03 medical and health sciences","Animals","Child, Preschool","Complement C1q","Complement Pathway, Classical","Disease Models, Animal","Humans","Male","Mice","Mice, Inbred C57BL","Microglia","Motor Neurons","Muscular Atrophy, Spinal","Synapses"],"mesh_terms":["Animals","Child, Preschool","Complement Pathway, Classical","Disease Models, Animal","Humans","Male","Mice, Inbred C57BL","Motor Neurons","Muscular Atrophy, Spinal","Synapses","Complement C1q","Microglia","Mice"],"keywords":["SMA*","Neuroscience","Microglia","Complement (music)","Spinal muscular atrophy","Biology","Motor neuron","Cell biology","Spinal cord","Inflammation","Immunology","Phenotype","Gene","Genetics","Computer science","Synapse","C1q","Smn","Classical Complement Pathway","Sensory-motor Circuit","Proprioceptive Neuron","Male","Motor 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