Cargando…

Human Induced Pluripotent Stem Cell-Derived TDP-43 Mutant Neurons Exhibit Consistent Functional Phenotypes Across Multiple Gene Edited Lines Despite Transcriptomic and Splicing Discrepancies

Gene editing technologies hold great potential to enhance our ability to model inheritable neurodegenerative diseases. Specifically, engineering multiple amyotrophic lateral sclerosis (ALS) mutations into isogenic cell populations facilitates determination of whether different causal mutations cause...

Descripción completa

Detalles Bibliográficos
Autores principales:	Smith, Alec S. T., Chun, Changho, Hesson, Jennifer, Mathieu, Julie, Valdmanis, Paul N., Mack, David L., Choi, Byung-Ok, Kim, Deok-Ho, Bothwell, Mark
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Frontiers Media S.A. 2021
Materias:	Cell and Developmental Biology
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8511491/ https://www.ncbi.nlm.nih.gov/pubmed/34660586 http://dx.doi.org/10.3389/fcell.2021.728707

Ejemplares similares

A C-Terminally Truncated TDP-43 Splice Isoform Exhibits Neuronal Specific Cytoplasmic Aggregation and Contributes to TDP-43 Pathology in ALS
por: Shenouda, Marc, et al.
Publicado: (2022)

Mice with endogenous TDP‐43 mutations exhibit gain of splicing function and characteristics of amyotrophic lateral sclerosis
por: Fratta, Pietro, et al.
Publicado: (2018)

ALS-linked TDP-43(M337V) knock-in mice exhibit splicing deregulation without neurodegeneration
por: Watanabe, Seiji, et al.
Publicado: (2020)

Quantitative analysis of cryptic splicing associated with TDP-43 depletion
por: Humphrey, Jack, et al.
Publicado: (2017)

The N-terminal dimerization is required for TDP-43 splicing activity
por: Jiang, Lei-Lei, et al.
Publicado: (2017)

Phase separation-deficient TDP43 remains functional in splicing
por: Schmidt, Hermann Broder, et al.
Publicado: (2019)

SUMOylation Regulates TDP-43 Splicing Activity and Nucleocytoplasmic Distribution
por: Maraschi, AnnaMaria, et al.
Publicado: (2021)

TDP-43 and Limbic-Predominant Age-Related TDP-43 Encephalopathy
por: Zhang, Lumi, et al.
Publicado: (2020)

DCTN1 Binds to TDP-43 and Regulates TDP-43 Aggregation
por: Deshimaru, Manami, et al.
Publicado: (2021)

Cross-Regulation between TDP-43 and Paraspeckles Promotes Pluripotency-Differentiation Transition
por: Modic, Miha, et al.
Publicado: (2019)

Disease‐linked TDP‐43 hyperphosphorylation suppresses TDP‐43 condensation and aggregation
por: Gruijs da Silva, Lara A, et al.
Publicado: (2022)

Rodent Models of TDP-43 Proteinopathy: Investigating the Mechanisms of TDP-43-Mediated Neurodegeneration
por: Gendron, Tania F., et al.
Publicado: (2011)

Chaperone Mediated Autophagy Degrades TDP-43 Protein and Is Affected by TDP-43 Aggregation
por: Ormeño, Fernando, et al.
Publicado: (2020)

Lack of association between TDP-43 pathology and tau mis-splicing in Alzheimer's disease
por: Niblock, Michael, et al.
Publicado: (2016)

Cell senescence, loss of splicing, and lipid metabolism in TDP-43-related neurodegenerative processes
por: Torres, Pascual, et al.
Publicado: (2023)

TDP-43 crosses the divide
por: Leslie, Mitch
Publicado: (2015)

Cytoplasmic mislocalization of RNA splicing factors and aberrant neuronal gene splicing in TDP-43 transgenic pig brain
por: Wang, Guohao, et al.
Publicado: (2015)

TDP-43: From Alzheimer’s Disease to Limbic-Predominant Age-Related TDP-43 Encephalopathy
por: Huang, Wendi, et al.
Publicado: (2020)

TDP-43 mislocalization drives neurofilament changes in a novel model of TDP-43 proteinopathy
por: Atkinson, Rachel, et al.
Publicado: (2021)

The cooperative binding of TDP-43 to GU-rich RNA repeats antagonizes TDP-43 aggregation
por: Rengifo-Gonzalez, Juan Carlos, et al.
Publicado: (2021)

Aggregation-prone TDP-43 sequesters and drives pathological transitions of free nuclear TDP-43
por: Keating, Sean S., et al.
Publicado: (2023)

Evidence of cerebellar TDP-43 loss of function in FTLD-TDP
por: Pickles, Sarah, et al.
Publicado: (2022)

Antibody against TDP-43 phosphorylated at serine 375 suggests conformational differences of TDP-43 aggregates among FTLD–TDP subtypes
por: Neumann, Manuela, et al.
Publicado: (2020)

The Pathological Phenotypes of Human TDP-43 Transgenic Mouse Models Are Independent of Downregulation of Mouse Tdp-43
por: Xu, Ya-Fei, et al.
Publicado: (2013)

TDP-43 and Phosphorylated TDP-43 Levels in Paired Plasma and CSF Samples in Amyotrophic Lateral Sclerosis
por: Ren, Yuting, et al.
Publicado: (2021)

TREM2 interacts with TDP-43 and mediates microglial neuroprotection against TDP-43-related neurodegeneration
por: Xie, Manling, et al.
Publicado: (2021)

Monomerization of TDP-43 is a key determinant for inducing TDP-43 pathology in amyotrophic lateral sclerosis
por: Oiwa, Kotaro, et al.
Publicado: (2023)

Characterising the RNA targets and position-dependent splicing regulation by TDP-43; implications for neurodegenerative diseases
por: Tollervey, James R., et al.
Publicado: (2011)

TDP-43 regulates global translational yield by splicing of exon junction complex component SKAR
por: Fiesel, Fabienne C., et al.
Publicado: (2012)

Alteration of POLDIP3 Splicing Associated with Loss of Function of TDP-43 in Tissues Affected with ALS
por: Shiga, Atsushi, et al.
Publicado: (2012)

High-Resolution RNA Maps Suggest Common Principles of Splicing and Polyadenylation Regulation by TDP-43
por: Rot, Gregor, et al.
Publicado: (2017)

TDP-43 regulates GAD1 mRNA splicing and GABA signaling in Drosophila CNS
por: Romano, Giulia, et al.
Publicado: (2021)

Mis-spliced transcripts generate de novo proteins in TDP-43-related ALS/FTD
por: Seddighi, Sahba, et al.
Publicado: (2023)

A fluid biomarker reveals loss of TDP-43 splicing repression in pre-symptomatic ALS
por: Irwin, Katherine E., et al.
Publicado: (2023)

Molecular Neuropathology of TDP-43 Proteinopathies
por: Neumann, Manuela
Publicado: (2009)

Mechanisms and models of TDP-43 proteinopathies
por: Petrucelli, Leonard
Publicado: (2012)

Drosophila Answers to TDP-43 Proteinopathies
por: Romano, Maurizio, et al.
Publicado: (2012)

Metals in ALS TDP-43 Pathology
por: Koski, Lassi, et al.
Publicado: (2021)

TDP-43 Pathology in Alzheimer’s Disease
por: Meneses, Axel, et al.
Publicado: (2021)

Divergent Phenotypes in Mutant TDP-43 Transgenic Mice Highlight Potential Confounds in TDP-43 Transgenic Modeling
por: D’Alton, Simon, et al.
Publicado: (2014)

Cannot write session to /tmp/vufind_sessions/sess_qc8md8f03j15em8ih729tl9b74