Improvement of cerebellar ataxic gait by injecting Cbln1 into the cerebellum of cbln1-null mice

Abstract Patients and rodents with cerebellar damage display ataxic gaits characterized by impaired coordination of limb movements. Here, gait ataxia in mice with a null mutation of the gene for the cerebellin 1 precursor protein (cbln1-null mice) was investigated by kinematic analysis of hindlimb m...

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Autores principales:	Eri Takeuchi, Aya Ito-Ishida, Michisuke Yuzaki, Dai Yanagihara
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2018
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/11de74f53cf24782ae498ca9920ca840
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spelling	oai:doaj.org-article:11de74f53cf24782ae498ca9920ca8402021-12-02T15:07:44ZImprovement of cerebellar ataxic gait by injecting Cbln1 into the cerebellum of cbln1-null mice10.1038/s41598-018-24490-02045-2322https://doaj.org/article/11de74f53cf24782ae498ca9920ca8402018-04-01T00:00:00Zhttps://doi.org/10.1038/s41598-018-24490-0https://doaj.org/toc/2045-2322Abstract Patients and rodents with cerebellar damage display ataxic gaits characterized by impaired coordination of limb movements. Here, gait ataxia in mice with a null mutation of the gene for the cerebellin 1 precursor protein (cbln1-null mice) was investigated by kinematic analysis of hindlimb movements during locomotion. The Cbln1 protein is predominately produced and secreted from cerebellar granule cells. The cerebellum of cbln1-null mice is characterized by an 80% reduction in the number of parallel fiber-Purkinje cell synapses compared with wild-type mice. Our analyses identified prominent differences in the temporal parameters of locomotion between cbln1-null and wild-type mice. The cbln1-null mice displayed abnormal hindlimb movements that were characterized by excessive toe elevation during the swing phase, and by severe hyperflexion of the ankles and knees. When recombinant Cbln1 protein was injected into the cerebellum of cbln1-null mice, the step cycle and stance phase durations increased toward those of wild-type mice, and the angular excursions of the knee during a cycle period showed a much closer agreement with those of wild-type mice. These findings suggest that dysfunction of the parallel fiber-Purkinje cell synapses might underlie the impairment of hindlimb movements during locomotion in cbln1-null mice.Eri TakeuchiAya Ito-IshidaMichisuke YuzakiDai YanagiharaNature PortfolioarticleMedicineRScienceQENScientific Reports, Vol 8, Iss 1, Pp 1-10 (2018)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Eri Takeuchi Aya Ito-Ishida Michisuke Yuzaki Dai Yanagihara Improvement of cerebellar ataxic gait by injecting Cbln1 into the cerebellum of cbln1-null mice
description	Abstract Patients and rodents with cerebellar damage display ataxic gaits characterized by impaired coordination of limb movements. Here, gait ataxia in mice with a null mutation of the gene for the cerebellin 1 precursor protein (cbln1-null mice) was investigated by kinematic analysis of hindlimb movements during locomotion. The Cbln1 protein is predominately produced and secreted from cerebellar granule cells. The cerebellum of cbln1-null mice is characterized by an 80% reduction in the number of parallel fiber-Purkinje cell synapses compared with wild-type mice. Our analyses identified prominent differences in the temporal parameters of locomotion between cbln1-null and wild-type mice. The cbln1-null mice displayed abnormal hindlimb movements that were characterized by excessive toe elevation during the swing phase, and by severe hyperflexion of the ankles and knees. When recombinant Cbln1 protein was injected into the cerebellum of cbln1-null mice, the step cycle and stance phase durations increased toward those of wild-type mice, and the angular excursions of the knee during a cycle period showed a much closer agreement with those of wild-type mice. These findings suggest that dysfunction of the parallel fiber-Purkinje cell synapses might underlie the impairment of hindlimb movements during locomotion in cbln1-null mice.
format	article
author	Eri Takeuchi Aya Ito-Ishida Michisuke Yuzaki Dai Yanagihara
author_facet	Eri Takeuchi Aya Ito-Ishida Michisuke Yuzaki Dai Yanagihara
author_sort	Eri Takeuchi
title	Improvement of cerebellar ataxic gait by injecting Cbln1 into the cerebellum of cbln1-null mice
title_short	Improvement of cerebellar ataxic gait by injecting Cbln1 into the cerebellum of cbln1-null mice
title_full	Improvement of cerebellar ataxic gait by injecting Cbln1 into the cerebellum of cbln1-null mice
title_fullStr	Improvement of cerebellar ataxic gait by injecting Cbln1 into the cerebellum of cbln1-null mice
title_full_unstemmed	Improvement of cerebellar ataxic gait by injecting Cbln1 into the cerebellum of cbln1-null mice
title_sort	improvement of cerebellar ataxic gait by injecting cbln1 into the cerebellum of cbln1-null mice
publisher	Nature Portfolio
publishDate	2018
url	https://doaj.org/article/11de74f53cf24782ae498ca9920ca840
work_keys_str_mv	AT eritakeuchi improvementofcerebellarataxicgaitbyinjectingcbln1intothecerebellumofcbln1nullmice AT ayaitoishida improvementofcerebellarataxicgaitbyinjectingcbln1intothecerebellumofcbln1nullmice AT michisukeyuzaki improvementofcerebellarataxicgaitbyinjectingcbln1intothecerebellumofcbln1nullmice AT daiyanagihara improvementofcerebellarataxicgaitbyinjectingcbln1intothecerebellumofcbln1nullmice
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Improvement of cerebellar ataxic gait by injecting Cbln1 into the cerebellum of cbln1-null mice

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