{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,11]],"date-time":"2026-02-11T21:14:16Z","timestamp":1770844456876,"version":"3.50.1"},"reference-count":48,"publisher":"MDPI AG","issue":"8","license":[{"start":{"date-parts":[[2022,4,14]],"date-time":"2022-04-14T00:00:00Z","timestamp":1649894400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["SCHA 185\/87-3"],"award-info":[{"award-number":["SCHA 185\/87-3"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Li Kashing Foundation","award":["none"],"award-info":[{"award-number":["none"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Adhesion molecules regulate cell proliferation, migration, survival, neuritogenesis, synapse formation and synaptic plasticity during the nervous system\u2019s development and in the adult. Among such molecules, the neural cell adhesion molecule L1 contributes to these functions during development, and in synapse formation, synaptic plasticity and regeneration after trauma. Proteolytic cleavage of L1 by different proteases is essential for these functions. A proteolytic fragment of 70 kDa (abbreviated L1-70) comprising part of the extracellular domain and the transmembrane and intracellular domains was shown to interact with mitochondrial proteins and is suggested to be involved in mitochondrial functions. To further determine the role of L1-70 in mitochondria, we generated two lines of gene-edited mice expressing full-length L1, but no or only low levels of L1-70. We showed that in the absence of L1-70, mitochondria in cultured cerebellar neurons move more retrogradely and exhibit reduced mitochondrial membrane potential, impaired Complex I activity and lower ATP levels compared to wild-type littermates. Neither neuronal migration, neuronal survival nor neuritogenesis in these mutants were stimulated with a function-triggering L1 antibody or with small agonistic L1 mimetics. These results suggest that L1-70 is important for mitochondrial homeostasis and that its absence contributes to the L1 syndrome phenotypes.<\/jats:p>","DOI":"10.3390\/ijms23084337","type":"journal-article","created":{"date-parts":[[2022,4,14]],"date-time":"2022-04-14T21:44:06Z","timestamp":1649972646000},"page":"4337","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Mitochondrial and Neuronal Dysfunctions in L1 Mutant Mice"],"prefix":"10.3390","volume":"23","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0657-1704","authenticated-orcid":false,"given":"Ludovica","family":"Congiu","sequence":"first","affiliation":[{"name":"Zentrum f\u00fcr Molekulare Neurobiologie, Universit\u00e4tsklinikum Hamburg-Eppendorf, Falkenried 94, 20251 Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8150-4889","authenticated-orcid":false,"given":"Viviana","family":"Granato","sequence":"additional","affiliation":[{"name":"Zentrum f\u00fcr Molekulare Neurobiologie, Universit\u00e4tsklinikum Hamburg-Eppendorf, Falkenried 94, 20251 Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gabriele","family":"Loers","sequence":"additional","affiliation":[{"name":"Zentrum f\u00fcr Molekulare Neurobiologie, Universit\u00e4tsklinikum Hamburg-Eppendorf, Falkenried 94, 20251 Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ralf","family":"Kleene","sequence":"additional","affiliation":[{"name":"Zentrum f\u00fcr Molekulare Neurobiologie, Universit\u00e4tsklinikum Hamburg-Eppendorf, Falkenried 94, 20251 Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Melitta","family":"Schachner","sequence":"additional","affiliation":[{"name":"Keck Center for Collaborative Neuroscience, Department of Cell Biology and Neuroscience, Rutgers University, Piscataway, NJ 08554, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,4,14]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"599792","DOI":"10.3389\/fcell.2020.599792","article-title":"Dysfunction of mitochondrial Ca(2+) regulatory machineries in brain aging and neurodegenerative diseases","volume":"8","author":"Jung","year":"2020","journal-title":"Front. 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