Cerebellum-enriched protein INPP5A contributes to selective neuropathology in mouse model of spinocerebellar ataxias type 17
oleh: Qiong Liu, Shanshan Huang, Peng Yin, Su Yang, Jennifer Zhang, Liang Jing, Siying Cheng, Beisha Tang, Xiao-Jiang Li, Yongcheng Pan, Shihua Li
| Format: | Article |
|---|---|
| Diterbitkan: | Nature Portfolio 2020-02-01 |
Deskripsi
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.