Explanipedia

Reduction of neuroinflammation and seizures in a mouse model of CLN1 batten disease using the small molecule enzyme mimetic, N-Tert-butyl hydroxylamine Open

Zach Fyke, Rachel Johansson, Anna I. Scott, Devin Wiley, Daniel Chelsky , et al. · 2024

Infantile neuronal ceroid lipofuscinosis (CLN1 Batten Disease) is a devastating pediatric lysosomal storage disease caused by pathogenic variants in the CLN1 gene, which encodes the depalmitoylation enzyme, palmitoyl-protein thioesterase 1…

Akap5 links synaptic dysfunction to neuroinflammatory signaling in a mouse model of infantile neuronal ceroid lipofuscinosis Open

Kevin P. Koster, Zach Fyke, Thu T. A. Nguyen, Amanda Niqula, Lorena Y. Noriega-González , et al. · 2024

Palmitoylation and depalmitoylation represent dichotomic processes by which a labile posttranslational lipid modification regulates protein trafficking and degradation. The depalmitoylating enzyme, palmitoyl-protein thioesterase 1 (PPT1), …

Loss of Depalmitoylation Disrupts Homeostatic Plasticity of AMPARs in a Mouse Model of Infantile Neuronal Ceroid Lipofuscinosis Open

Kevin P. Koster, Edén Flores-Barrera, Emilce Artur de la Villarmois, Adriana Caballero, Kuei Y. Tseng , et al. · 2023

Protein palmitoylation is the only reversible post-translational lipid modification. Palmitoylation is held in delicate balance by depalmitoylation to precisely regulate protein turnover. While over 20 palmitoylation enzymes are known, dep…

Commensal-specific CD4 T cells promote inflammation in the central nervous system via molecular mimicry Open

Zachary White, Ivan Cabrera, Andrea Ochoa-Raya, Isabel Kapustka, Kevin P. Koster , et al. · 2022

Commensal bacteria are critical regulators of both tissue homeostasis and the development and exacerbation of autoimmunity. However, it remains unclear how the intestinal microbiota contributes to inflammation in tissues such as the centra…

Loss of Depalmitoylation Exaggerates Synaptic Upscaling and Leads to Neuroinflammation in a Lysosomal Storage Disease Open

Kevin P. Koster, Edén Flores-Barrera, Emilce Artur de la Villarmois, Thu T. A. Nguyen, Amanda Niqula , et al. · 2021

Summary Palmitoylation and depalmitoylation are the dichotomic processes of lipid modification regulating protein trafficking, recycling, and degradation, thereby controlling proteostasis. Despite our understanding of palmitoylation, depal…

A novel TSC1 variant associated with tuberous sclerosis and sacrococcygeal teratoma Open

Saba Ahmad, Luis Manon, Gifty Bhat, Jerry Machado, Alice Zalan , et al. · 2020

Editorial: Role of Protein Palmitoylation in Synaptic Plasticity and Neuronal Differentiation Open

Akira Yoshii, William N. Green · 2020

© The Author(s), 2020. This article is distributed under the terms of the Creative Commons Attribution License. The definitive version was published in Yoshii, A., & Green, W. N. Editorial: role of protein palmitoylation in synaptic plasti…

Depalmitoylation by Palmitoyl-Protein Thioesterase 1 in Neuronal Health and Degeneration Open

Kevin P. Koster, Akira Yoshii · 2019

Protein palmitoylation is the post-translational, reversible addition of a 16-carbon fatty acid, palmitate, to proteins. Protein palmitoylation has recently garnered much attention, as it robustly modifies the localization and function of …

Developmental NMDA receptor dysregulation in the infantile neuronal ceroid lipofuscinosis mouse model Open

Kevin P. Koster, Walter Francesconi, Fulvia Berton, Sami Alahmadi, Roshan Srinivas , et al. · 2019

Protein palmitoylation and depalmitoylation alter protein function. This post-translational modification is critical for synaptic transmission and plasticity. Mutation of the depalmitoylating enzyme palmitoyl-protein thioesterase 1 (PPT1) …

Hyperexcitability of the local cortical circuit in mouse models of tuberous sclerosis complex Open

Jianping Zhao, Akira Yoshii · 2019

Author response: Developmental NMDA receptor dysregulation in the infantile neuronal ceroid lipofuscinosis mouse model Open

Kevin P. Koster, Walter Francesconi, Fulvia Berton, Sami Alahmadi, Roshan Srinivas , et al. · 2018

Multiple Critical Periods for Rapamycin Treatment to Correct Structural Defects in Tsc-1-Suppressed Brain Open

Rebecca L. Cox, Froylán Calderón de Anda, Tomer Mangoubi, Akira Yoshii · 2018

Tuberous sclerosis complex (TSC) is an autosomal dominant neurogenetic disorder affecting the brain and other vital organs. Neurological symptoms include epilepsy, intellectual disability, and autism. TSC is caused by a loss-of-function mu…

NMDA Receptor Dysregulation by Defective Depalmitoylation in the Infantile Neuronal Ceroid Lipofuscinosis Mouse Model Open

Kevin P. Koster, Walter Francesconi, Fulvia Berton, Sami Alahmadi, Roshan Srinivas , et al. · 2018

Protein palmitoylation and depalmitoylation alter protein function. This post-translational modification is critical for synaptic transmission and plasticity. Mutation of the depalmitoylating enzyme palmitoyl-protein thioesterase 1 (PPT1) …

Editorial: Cell and molecular signaling, and transport pathways involved in growth factor control of synaptic development and function Open

Akira Yoshii, Martha Constantine‐Paton, Nancy Y. Ip · 2015

Since the discovery of nerve growth factor (NGF) more than a half century ago (Levi-Montalcini and Cohen, 1960), the prototypic neurotrophin family has included brain derived neurotrophic factor (BDNF), neurotrophin-3 (NT-3), and neurotrop…

Cell and Molecular Signaling, and Transport Pathways Involved in Growth Factor Control of Synaptic Development and Function Open

Akira Yoshii, Martha Constantine‐Paton, Nancy Y. Ip · 2015

EDITORIAL article Front. Synaptic Neurosci., 04 June 2015 Volume 7 - 2015 | https://doi.org/10.3389/fnsyn.2015.00008

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