{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Biundo F"],"funding":["NICHD NIH HHS","Sol Goldman Charitable Trust","NIA NIH HHS","NIMH NIH HHS","NINDS NIH HHS","NCI NIH HHS","National Institutes of Health","NIH HHS"],"pagination":["775-794"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC9868112"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["71(3)"],"pubmed_abstract":["Colony stimulating factor (CSF) receptor-1 (CSF-1R)-related leukoencephalopathy (CRL) is an adult-onset, demyelinating and neurodegenerative disease caused by autosomal dominant mutations in CSF1R, modeled by the Csf1r<sup>+/-</sup> mouse. The expression of Csf2, encoding granulocyte-macrophage CSF (GM-CSF) and of Csf3, encoding granulocyte CSF (G-CSF), are elevated in both mouse and human CRL brains. While monoallelic targeting of Csf2 has been shown to attenuate many behavioral and histological deficits of Csf1r<sup>+/-</sup> mice, including cognitive dysfunction and demyelination, the contribution of Csf3 has not been explored. In the present study, we investigate the behavioral, electrophysiological and histopathological phenotypes of Csf1r<sup>+/-</sup> mice following monoallelic targ"],"journal":["Glia"],"pubmed_title":["Elevated granulocyte colony stimulating factor (CSF) causes cerebellar deficits and anxiety in a model of CSF-1 receptor related leukodystrophy."],"pmcid":["PMC9868112"],"funding_grant_id":["U54 HD090260","1U19AG063911","R01 NS105470","P30CA013330","P50 HD105352","R01NS091519","R01 NS091519","R21 MH114182","P30 CA013330","R01NS105470","U19AG063911","R21MH114182","U19 AG063911"],"pubmed_authors":["Burghardt NS","Dickson DW","Biundo F","Wszolek ZK","DeTure MA","Shlager GGL","Stanley ER","Ketchum HC","Chitu V","Khodakhah K","Tindi J"],"additional_accession":[]},"is_claimable":false,"name":"Elevated granulocyte colony stimulating factor (CSF) causes cerebellar deficits and anxiety in a model of CSF-1 receptor related leukodystrophy.","description":"Colony stimulating factor (CSF) receptor-1 (CSF-1R)-related leukoencephalopathy (CRL) is an adult-onset, demyelinating and neurodegenerative disease caused by autosomal dominant mutations in CSF1R, modeled by the Csf1r<sup>+/-</sup> mouse. The expression of Csf2, encoding granulocyte-macrophage CSF (GM-CSF) and of Csf3, encoding granulocyte CSF (G-CSF), are elevated in both mouse and human CRL brains. While monoallelic targeting of Csf2 has been shown to attenuate many behavioral and histological deficits of Csf1r<sup>+/-</sup> mice, including cognitive dysfunction and demyelination, the contribution of Csf3 has not been explored. In the present study, we investigate the behavioral, electrophysiological and histopathological phenotypes of Csf1r<sup>+/-</sup> mice following monoallelic targ","dates":{"release":"2023-01-01T00:00:00Z","publication":"2023 Mar","modification":"2026-06-01T17:22:33.221Z","creation":"2025-04-03T23:50:17.557Z"},"accession":"S-EPMC9868112","cross_references":{"pubmed":["36433736"],"doi":["10.1002/glia.24310"]}}