Cerebellum-enriched protein INPP5A contributes to selective neuropathology in mouse model of spinocerebellar ataxias type 17

It is not yet clear how ubiquitously-expressed proteins can cause the selective degeneration of particular populations of neurons, such as in spinocerebellar ataxia type 17, SCA17, which results from a CAG trinucleotide repeat expansion in the ubiquitously expressed transcription factor TBP. Here, t...

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Autores principales:	Qiong Liu, Shanshan Huang, Peng Yin, Su Yang, Jennifer Zhang, Liang Jing, Siying Cheng, Beisha Tang, Xiao-Jiang Li, Yongcheng Pan, Shihua Li
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2020
Materias:	Science Q
Acceso en línea:	https://doaj.org/article/2411a8058950408fae8d5c6dceaba888
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spelling	oai:doaj.org-article:2411a8058950408fae8d5c6dceaba8882021-12-02T15:33:30ZCerebellum-enriched protein INPP5A contributes to selective neuropathology in mouse model of spinocerebellar ataxias type 1710.1038/s41467-020-14931-82041-1723https://doaj.org/article/2411a8058950408fae8d5c6dceaba8882020-02-01T00:00:00Zhttps://doi.org/10.1038/s41467-020-14931-8https://doaj.org/toc/2041-1723It is not yet clear how ubiquitously-expressed proteins can cause the selective degeneration of particular populations of neurons, such as in spinocerebellar ataxia type 17, SCA17, which results from a CAG trinucleotide repeat expansion in the ubiquitously expressed transcription factor TBP. Here, the authors show that mutant TBP suppresses the cerebellum-enriched transcription of Inpp5a and link altered levels of INPP5A to the selective degeneration of cerebellar neurons.Qiong LiuShanshan HuangPeng YinSu YangJennifer ZhangLiang JingSiying ChengBeisha TangXiao-Jiang LiYongcheng PanShihua LiNature PortfolioarticleScienceQENNature Communications, Vol 11, Iss 1, Pp 1-13 (2020)
institution	DOAJ
collection	DOAJ
language	EN
topic	Science Q
spellingShingle	Science Q Qiong Liu Shanshan Huang Peng Yin Su Yang Jennifer Zhang Liang Jing Siying Cheng Beisha Tang Xiao-Jiang Li Yongcheng Pan Shihua Li Cerebellum-enriched protein INPP5A contributes to selective neuropathology in mouse model of spinocerebellar ataxias type 17
description	It is not yet clear how ubiquitously-expressed proteins can cause the selective degeneration of particular populations of neurons, such as in spinocerebellar ataxia type 17, SCA17, which results from a CAG trinucleotide repeat expansion in the ubiquitously expressed transcription factor TBP. Here, the authors show that mutant TBP suppresses the cerebellum-enriched transcription of Inpp5a and link altered levels of INPP5A to the selective degeneration of cerebellar neurons.
format	article
author	Qiong Liu Shanshan Huang Peng Yin Su Yang Jennifer Zhang Liang Jing Siying Cheng Beisha Tang Xiao-Jiang Li Yongcheng Pan Shihua Li
author_facet	Qiong Liu Shanshan Huang Peng Yin Su Yang Jennifer Zhang Liang Jing Siying Cheng Beisha Tang Xiao-Jiang Li Yongcheng Pan Shihua Li
author_sort	Qiong Liu
title	Cerebellum-enriched protein INPP5A contributes to selective neuropathology in mouse model of spinocerebellar ataxias type 17
title_short	Cerebellum-enriched protein INPP5A contributes to selective neuropathology in mouse model of spinocerebellar ataxias type 17
title_full	Cerebellum-enriched protein INPP5A contributes to selective neuropathology in mouse model of spinocerebellar ataxias type 17
title_fullStr	Cerebellum-enriched protein INPP5A contributes to selective neuropathology in mouse model of spinocerebellar ataxias type 17
title_full_unstemmed	Cerebellum-enriched protein INPP5A contributes to selective neuropathology in mouse model of spinocerebellar ataxias type 17
title_sort	cerebellum-enriched protein inpp5a contributes to selective neuropathology in mouse model of spinocerebellar ataxias type 17
publisher	Nature Portfolio
publishDate	2020
url	https://doaj.org/article/2411a8058950408fae8d5c6dceaba888
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Cerebellum-enriched protein INPP5A contributes to selective neuropathology in mouse model of spinocerebellar ataxias type 17

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