Publications by authors named "Moritz J Frech"

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Disrupted Myelination in FAHN: Insights from a Patient-Specific hiPSC Neuron-Oligodendrocyte Model.
Fatima Efendic, Andreas Hermann, Moritz J Frech

Cells· August 2025

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Generation of the human iPSC lines AKOSi011-A carrying the mutation p.Pro65Ser/p.Asp35T and AKOSi012-A, carrying the mutation p.Tyr231His, derived from FAHN patient fibroblasts.
Fatima Efendic, Saskia Krohn, Hugo Murua Escobar, Sunita Venkateswaran, Steffany A L Bennett, Moritz J Frech

Stem Cell Res· September 2023

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FUS ALS neurons activate major stress pathways and reduce translation as an early protective mechanism against neurodegeneration.
Barbara Szewczyk, René Günther, Julia Japtok, Moritz J Frech, Marcel Naumann

Cell Rep· February 2023

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Generation of the human iPSC line AKOSi010-A from fibroblasts of a female FAHN patient, carrying the compound heterozygous mutation p.Gly45Arg/p.His319Arg.
Fatima Efendic, Christin Völkner, Saskia Krohn, Hugo Murua Escobar, Sunita Venkateswaran, Moritz J Frech

Stem Cell Res· August 2022

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Impact of Organelle Transport Deficits on Mitophagy and Autophagy in Niemann-Pick Disease Type C.
Maik Liedtke, Christin Völkner, Andreas Hermann, Moritz J Frech

Cells· February 2022

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Assessment of FDA-Approved Drugs as a Therapeutic Approach for Niemann-Pick Disease Type C1 Using Patient-Specific iPSC-Based Model Systems.
Christin Völkner, Supansa Pantoom, Maik Liedtke, Jan Lukas, Andreas Hermann, Moritz J Frech

Cells· January 2022

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Patient-Specific iPSC-Derived Neural Differentiated and Hepatocyte-like Cells, Carrying the Compound Heterozygous Mutation p.V1023Sfs*15/p.G992R, Present the "Variant" Biochemical Phenotype of Niemann-Pick Type C1 Disease.
Christin Völkner, Maik Liedtke, Robert Untucht, Andreas Hermann, Moritz J Frech

Int J Mol Sci· November 2021

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Pathophysiological In Vitro Profile of Neuronal Differentiated Cells Derived from Niemann-Pick Disease Type C2 Patient-Specific iPSCs Carrying the Mutations c.58G>T/c.140G>T.
Maik Liedtke, Christin Völkner, Alexandra V Jürs, Franziska Peter, Michael Rabenstein, Moritz J Frech

Int J Mol Sci· April 2021

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Pluripotent Stem Cells for Disease Modeling and Drug Discovery in Niemann-Pick Type C1.
Christin Völkner, Maik Liedtke, Andreas Hermann, Moritz J Frech

Int J Mol Sci· January 2021

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Generation of an iPSC line (AKOSi006-A) from fibroblasts of an NPC1 patient, carrying the homozygous mutation p.I1061T (c.3182 T > C) and a control iPSC line (AKOSi007-A) using a non-integrating Sendai virus system.
Christin Völkner, Maik Liedtke, Janine Petters, Katharina Huth, Gudrun Knuebel, Moritz J Frech

Stem Cell Res· December 2020

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Generation of two induced pluripotent stem cell lines from a female adult homozygous for the Wilson disease associated ATP7B variant p.H1069Q (AKOSi008-A) and a healthy control (AKOSi009-A).
Janine Petters, Christin Völkner, Saskia Krohn, Hugo Murua Escobar, Jörn Bullerdiek, Moritz J Frech

Stem Cell Res· December 2020

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Generation of an iPSC line (AKOSi004-A) from fibroblasts of a female adult NPC1 patient, carrying the compound heterozygous mutation p.Val1023Serfs*15/p.Gly992Arg and of an iPSC line (AKOSi005-A) from a female adult control individual.
Christin Völkner, Maik Liedtke, Janine Petters, Jan Lukas, Hugo Murua Escobar, Moritz J Frech

Stem Cell Res· December 2020

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Oxidative Stress and Alterations in the Antioxidative Defense System in Neuronal Cells Derived from NPC1 Patient-Specific Induced Pluripotent Stem Cells.
Alexandra V Jürs, Christin Völkner, Maik Liedtke, Katharina Huth, Jan Lukas, Moritz J Frech

Int J Mol Sci· October 2020

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Identification of Brain-Specific Treatment Effects in NPC1 Disease by Focusing on Cellular and Molecular Changes of Sphingosine-1-Phosphate Metabolism.
Anne Gläser, Franziska Hammerl, Markus H Gräler, Sina M Coldewey, Christin Völkner, Moritz J Frech

Int J Mol Sci· June 2020

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Stimulation of mGluR1/5 Improves Defective Internalization of AMPA Receptors in NPC1 Mutant Mouse.
Xiao Feng, Fan Yang, Michael Rabenstein, Zhen Wang, Moritz J Frech

Cereb Cortex· March 2020

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Generation of induced pluripotent stem cell lines AKOSi002-A and AKOSi003-A from symptomatic female adults with Wilson disease.
Janine Petters, Chiara Cimmaruta, Katharina Iwanov, Matthew L Chang, Christin Völkner, Moritz J Frech

Stem Cell Res· March 2020

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Generation of the Niemann-Pick type C2 patient-derived iPSC line AKOSi001-A.
Christin Völkner, Franziska Peter, Maik Liedtke, Saskia Krohn, Iris Lindner, Moritz J Frech

Stem Cell Res· December 2019

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Alteration of GABAergic Input Precedes Neurodegeneration of Cerebellar Purkinje Cells of NPC1-Deficient Mice.
Michael Rabenstein, Nico Murr, Andreas Hermann, Arndt Rolfs, Moritz J Frech

Int J Mol Sci· December 2019

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Determination of the Pathological Features of NPC1 Variants in a Cellular Complementation Test.
Xiao Feng, Claudia Cozma, Supansa Pantoom, Christina Hund, Katharina Iwanov, Moritz J Frech

Int J Mol Sci· October 2019

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Impact of Reduced Cerebellar EAAT Expression on Purkinje Cell Firing Pattern of NPC1-deficient Mice.
Michael Rabenstein, Franziska Peter, Arndt Rolfs, Moritz J Frech

Sci Rep· February 2018

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Decreased calcium flux in Niemann-Pick type C1 patient-specific iPSC-derived neurons due to higher amount of calcium-impermeable AMPA receptors.
Michael Rabenstein, Franziska Peter, Sarah Joost, Michaela Trilck, Arndt Rolfs, Moritz J Frech

Mol Cell Neurosci· September 2017

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Non-canonical pathway induced by Wnt3a regulates β-catenin via Pyk2 in differentiating human neural progenitor cells.
Venkata Ajay Narendra Talabattula, Peter Morgan, Moritz J Frech, Adelinde M Uhrmacher, Ottmar Herchenröder

Biochem Biophys Res Commun· September 2017

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Activation of PKC triggers rescue of NPC1 patient specific iPSC derived glial cells from gliosis.
Franziska Peter, Sebastian Rost, Arndt Rolfs, Moritz J Frech

Orphanet J Rare Dis· August 2017

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Dataset in support of the generation of Niemann-Pick disease Type C1 patient-specific iPS cell lines carrying the novel NPC1 mutation c.1180T>C or the prevalent c.3182T>C mutation - Analysis of pluripotency and neuronal differentiation.
Franziska Peter, Michaela Trilck, Michael Rabenstein, Arndt Rolfs, Moritz J Frech

Data Brief· June 2017

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Diversity of glycosphingolipid GM2 and cholesterol accumulation in NPC1 patient-specific iPSC-derived neurons.
Michaela Trilck, Franziska Peter, Chaonan Zheng, Marcus Frank, Kostantin Dobrenis, Moritz J Frech

Brain Res· February 2017

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