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Adv."],"published-print":{"date-parts":[[2024,7,26]]},"abstract":"<jats:p>\n            Mutations in Dystonin (\n            <jats:italic>DST<\/jats:italic>\n            ), which encodes cytoskeletal linker proteins, cause hereditary sensory and autonomic neuropathy 6 (HSAN-VI) in humans and the\n            <jats:italic>dystonia musculorum<\/jats:italic>\n            (\n            <jats:italic>dt<\/jats:italic>\n            ) phenotype in mice; however, the neuronal circuit underlying the HSAN-VI and\n            <jats:italic>dt<\/jats:italic>\n            phenotype is unresolved.\n            <jats:italic>dt<\/jats:italic>\n            mice exhibit dystonic movements accompanied by the simultaneous contraction of agonist and antagonist muscles and postnatal lethality. Here, we identified the sensory-motor circuit as a major causative neural circuit using a gene trap system that enables neural circuit-selective inactivation and restoration of\n            <jats:italic>Dst<\/jats:italic>\n            by Cre-mediated recombination. Sensory neuron\u2013selective\n            <jats:italic>Dst<\/jats:italic>\n            deletion led to motor impairment, degeneration of proprioceptive sensory neurons, and disruption of the sensory-motor circuit. Restoration of\n            <jats:italic>Dst<\/jats:italic>\n            expression in sensory neurons using Cre driver mice or a single postnatal injection of Cre-expressing adeno-associated virus ameliorated sensory degeneration and improved abnormal movements. These findings demonstrate that the sensory-motor circuit is involved in the movement disorders in\n            <jats:italic>dt<\/jats:italic>\n            mice and that the sensory circuit is a therapeutic target for HSAN-VI.\n          <\/jats:p>","DOI":"10.1126\/sciadv.adj9335","type":"journal-article","created":{"date-parts":[[2024,7,26]],"date-time":"2024-07-26T17:58:28Z","timestamp":1722016708000},"update-policy":"https:\/\/doi.org\/10.34133\/aaas_crossmark","source":"Crossref","is-referenced-by-count":3,"title":["Sensory-motor circuit is a therapeutic target for\n            <i>dystonia musculorum<\/i>\n            mice, a model of hereditary sensory and autonomic neuropathy 6"],"prefix":"10.1126","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4425-5457","authenticated-orcid":true,"given":"Nozomu","family":"Yoshioka","sequence":"first","affiliation":[{"name":"Division of Neurobiology and Anatomy, Graduate School of Medical and Dental Sciences, Niigata University, Niigata, Japan."},{"name":"Transdisciplinary Research Programs, Niigata University, Niigata, Japan."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masayuki","family":"Kurose","sequence":"additional","affiliation":[{"name":"Department of Physiology, School of Dentistry, Iwate Medical University, Yahaba, Japan."},{"name":"Division of Oral Physiology, Graduate School of Medical and Dental Sciences, Niigata University, Niigata, Japan."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7347-8150","authenticated-orcid":true,"given":"Hiromi","family":"Sano","sequence":"additional","affiliation":[{"name":"Division of System Neurophysiology, National Institute for Physiological Sciences, Okazaki, Japan."},{"name":"Physiological Sciences, SOKENDAI, Okazaki, Japan."},{"name":"Division of Behavioral Neuropharmacology, International Center for Brain Science, Fujita Health University, Toyoake, Japan."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dang Minh","family":"Tran","sequence":"additional","affiliation":[{"name":"Division of Neurobiology and Anatomy, Graduate School of Medical and Dental Sciences, Niigata University, Niigata, Japan."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2679-9530","authenticated-orcid":true,"given":"Satomi","family":"Chiken","sequence":"additional","affiliation":[{"name":"Division of System Neurophysiology, National Institute for Physiological Sciences, Okazaki, Japan."},{"name":"Physiological Sciences, SOKENDAI, Okazaki, Japan."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kazuki","family":"Tainaka","sequence":"additional","affiliation":[{"name":"Department of System Pathology for Neurological Disorders, Brain Research Institute, Niigata University, Niigata, 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