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

Metabolic Deficits in the Retina of a Familial Dysautonomia Mouse Model

家族性自主神经功能紊乱小鼠模型的视网膜代谢缺陷

Stephanann M Costello, Anastasia Schultz, Donald Smith, Danielle Horan, Martha Chaverra, Brian Tripet, Lynn George, Brian Bothner, Frances Lefcort, Valérie Copié

Gut microbiome dysbiosis drives metabolic dysfunction in Familial dysautonomia

肠道微生物群失调导致家族性自主神经功能紊乱导致代谢功能障碍

Alexandra M Cheney #, Stephanann M Costello #, Nicholas V Pinkham #, Annie Waldum, Susan C Broadaway, Maria Cotrina-Vidal, Marc Mergy, Brian Tripet, Douglas J Kominsky, Heather M Grifka-Walk, Horacio Kaufmann, Lucy Norcliffe-Kaufmann, Jesse T Peach, Brian Bothner, Frances Lefcort, Valérie Copié, Set

Reduction of retinal ganglion cell death in mouse models of familial dysautonomia using AAV-mediated gene therapy and splicing modulators

使用 AAV 介导的基因治疗和剪接调节剂减少家族性自主神经功能紊乱小鼠模型中的视网膜神经节细胞死亡

Anastasia Schultz, Shun-Yun Cheng, Emily Kirchner, Stephanann Costello, Heini Miettinen, Marta Chaverra, Colin King, Lynn George, Xin Zhao, Jana Narasimhan, Marla Weetall, Susan Slaugenhaupt, Elisabetta Morini, Claudio Punzo, Frances Lefcort

Loss of Elp1 disrupts trigeminal ganglion neurodevelopment in a model of familial dysautonomia

在家族性自主神经功能紊乱模型中,Elp1 的缺失会破坏三叉神经节的神经发育

Carrie E Leonard, Jolie Quiros, Frances Lefcort, Lisa A Taneyhill

Elp1 is required for development of visceral sensory peripheral and central circuitry

Elp1 是内脏感觉外周和中枢回路发育所必需的

Zariah Tolman, Marta Chaverra, Lynn George, Frances Lefcort

Loss of Elp1 perturbs histone H2A.Z and the Notch signaling pathway

Elp1 的缺失会扰乱组蛋白 H2A.Z 和 Notch 信号通路

BreAnna Cameron, Elin Lehrmann, Tien Chih, Joseph Walters, Richard Buksch, Sara Snyder, Joy Goffena, Frances Lefcort, Kevin G Becker, Lynn George

Elongator and codon bias regulate protein levels in mammalian peripheral neurons

延伸因子和密码子偏好性调节哺乳动物外周神经元中的蛋白质水平

Joy Goffena ,Frances Lefcort ,Yongqing Zhang ,Elin Lehrmann ,Marta Chaverra ,Jehremy Felig ,Joseph Walters ,Richard Buksch ,Kevin G Becker ,Lynn George

Retina-specific loss of Ikbkap/Elp1 causes mitochondrial dysfunction that leads to selective retinal ganglion cell degeneration in a mouse model of familial dysautonomia

视网膜特异性 Ikbkap/Elp1 缺失引起线粒体功能障碍,导致家族性自主神经功能紊乱小鼠模型中选择性视网膜神经节细胞变性

Yumi Ueki, Veronika Shchepetkina, Frances Lefcort

The familial dysautonomia disease gene IKBKAP is required in the developing and adult mouse central nervous system

家族性自主神经功能紊乱疾病基因 IKBKAP 是小鼠发育和成年中枢神经系统所必需的

Marta Chaverra, Lynn George, Marc Mergy, Hannah Waller, Katharine Kujawa, Connor Murnion, Ezekiel Sharples, Julian Thorne, Nathaniel Podgajny, Andrea Grindeland, Yumi Ueki, Steven Eiger, Cassie Cusick, A Michael Babcock, George A Carlson, Frances Lefcort

In vivo time-lapse imaging reveals extensive neural crest and endothelial cell interactions during neural crest migration and formation of the dorsal root and sympathetic ganglia

体内延时成像揭示了神经嵴迁移和背根和交感神经节形成过程中广泛的神经嵴和内皮细胞相互作用

Lynn George, Haley Dunkel, Barbara J Hunnicutt, Michael Filla, Charles Little, Rusty Lansford, Frances Lefcort