日期:
2020 年 — 2026 年
2020
2021
2022
2023
2024
2025
2026
影响因子:

Dysregulation of zebrin-II cell subtypes in the cerebellum is a shared feature across polyglutamine ataxia mouse models and patients

小脑中 zebrin-II 细胞亚型的失调是多聚谷氨酰胺共济失调小鼠模型和患者的共同特征

Luke C Bartelt, Pawel M Switonski, Grażyna Adamek, Fabiana Longo, Juliana Carvalho, Lisa A Duvick, Sabrina I Jarrah, Hayley S McLoughlin, Daniel R Scoles, Stefan M Pulst, Harry T Orr, Court Hull, Craig B Lowe, Albert R La Spada0

Antibody-assisted selective isolation of Purkinje cell nuclei from mouse cerebellar tissue

抗体辅助从小鼠小脑组织中选择性分离浦肯野细胞核

Luke C Bartelt, Mouad Fakhri, Grazyna Adamek, Magdalena Trybus, Anna Samelak-Czajka, Paulina Jackowiak, Agnieszka Fiszer, Craig B Lowe, Albert R La Spada, Pawel M Switonski

Altered H3 histone acetylation impairs high-fidelity DNA repair to promote cerebellar degeneration in spinocerebellar ataxia type 7

组蛋白H3乙酰化异常会损害高保真DNA修复,从而促进脊髓小脑性共济失调7型的小脑变性

Pawel M Switonski ,Joe R Delaney ,Luke C Bartelt ,Chenchen Niu ,Maria Ramos-Zapatero ,Nathanael J Spann ,Akshay Alaghatta ,Toby Chen ,Emily N Griffin ,Jaidev Bapat ,Bryce L Sopher ,Albert R La Spada

Respiratory dysfunction in a mouse model of spinocerebellar ataxia type 7

脊髓小脑性共济失调 7 型小鼠模型的呼吸功能障碍

Anna F Fusco, Logan A Pucci, Pawel M Switonski, Debolina D Biswas, Angela L McCall, Amanda F Kahn, Justin S Dhindsa, Laura M Strickland, Albert R La Spada, Mai K ElMallah

Nicotinamide Pathway-Dependent Sirt1 Activation Restores Calcium Homeostasis to Achieve Neuroprotection in Spinocerebellar Ataxia Type 7

烟酰胺通路依赖的Sirt1激活可恢复钙稳态,从而在7型脊髓小脑性共济失调中发挥神经保护作用

Colleen A Stoyas ,David D Bushart ,Pawel M Switonski ,Jacqueline M Ward ,Akshay Alaghatta ,Mi-Bo Tang ,Chenchen Niu ,Mandheer Wadhwa ,Haoran Huang ,Alex Savchenko ,Karim Gariani ,Fang Xie ,Joseph R Delaney ,Terry Gaasterland ,Johan Auwerx ,Vikram G Shakkottai ,Albert R La Spada

Altered expression of CYP17A1 and CYP19A1 in undescended testes of dogs with unilateral cryptorchidism

单侧隐睾犬隐睾中 CYP17A1 和 CYP19A1 表达改变

P Krzeminska, M Stachowiak, M Skrzypski, T Nowak, A Maslak, M Switonski

Metabolic and Organelle Morphology Defects in Mice and Human Patients Define Spinocerebellar Ataxia Type 7 as a Mitochondrial Disease

小鼠和人类患者的代谢和细胞器形态缺陷将脊髓小脑共济失调 7 型定义为线粒体疾病

Jacqueline M Ward, Colleen A Stoyas, Pawel M Switonski, Farid Ichou, Weiwei Fan, Brett Collins, Christopher E Wall, Isaac Adanyeguh, Chenchen Niu, Bryce L Sopher, Chizuru Kinoshita, Richard S Morrison, Alexandra Durr, Alysson R Muotri, Ronald M Evans, Fanny Mochel, Albert R La Spada

A new humanized ataxin-3 knock-in mouse model combines the genetic features, pathogenesis of neurons and glia and late disease onset of SCA3/MJD

一种新的人源化 Ataxin-3 敲入小鼠模型结合了 SCA3/MJD 的遗传特征、神经元和神经胶质细胞的发病机制以及晚期发病

Pawel M Switonski, Wojciech J Szlachcic, Wlodzimierz J Krzyzosiak, Maciej Figiel

Huntington disease iPSCs show early molecular changes in intracellular signaling, the expression of oxidative stress proteins and the p53 pathway

亨廷顿病 iPSC 显示出细胞内信号传导、氧化应激蛋白表达和 p53 通路的早期分子变化

Wojciech J Szlachcic, Pawel M Switonski, Wlodzimierz J Krzyzosiak, Marek Figlerowicz, Maciej Figiel

An evaluation of oligonucleotide-based therapeutic strategies for polyQ diseases

基于寡核苷酸的 polyQ 疾病治疗策略评估

Agnieszka Fiszer, Marta Olejniczak, Pawel M Switonski, Joanna P Wroblewska, Joanna Wisniewska-Kruk, Agnieszka Mykowska, Wlodzimierz J Krzyzosiak