Patient iPSC-Derived Neurons for Disease Modeling of Frontotemporal Dementia with Mutation in CHMP2B.

doi:10.1016/j.stemcr.2017.01.012

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Community

Back to search

Article

Patient iPSC-Derived Neurons for Disease Modeling of Frontotemporal Dementia with Mutation in CHMP2B.

Stem cell reports - 14 Mar 2017

Zhang Yu, Schmid Benjamin, Nikolaisen Nanett K, Rasmussen Mikkel A, Aldana Blanca I, Agger Mikkel, Calloe Kirstine, Stummann Tina C, Larsen Hjalte M, Nielsen Troels T, Huang Jinrong, Xu Fengping, Liu Xin, Bolund Lars, Meyer Morten, Bak Lasse K, Waagepetersen Helle S, Luo Yonglun, Nielsen Jørgen E, Holst Bjørn, Clausen Christian, Hyttel Poul, Freude Kristine K

Abstract excerpt

The truncated mutant form of the charged multivesicular body protein 2B (CHMP2B) is causative for frontotemporal dementia linked to chromosome 3 (FTD3). CHMP2B is a constituent of the endosomal sorting complex required for transport (ESCRT) and, when mutated, disrupts endosome-to-lysosome trafficking and substrate degradation. To understand the underlying molecular pathology, FTD3 patient induced pluripotent stem...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Cellular Reprogramming

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Endosomal Sorting Complexes Required for Transport

Share to Endosomes

Share to Frontotemporal Dementia

Share to Induced Pluripotent Stem Cells

Share to Iron

Share to Oxidative Stress

Share to Transcriptome

Source

Open in PubMed Open full text

Identifiers

PMID: 28216144
DOI: 10.1016/j.stemcr.2017.01.012
PMCID: PMC5355623

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Patient iPSC-Derived Neurons for Disease Modeling of Frontotemporal Dementia with Mutation in CHMP2B.PMID 28216144Read ↗

Patient-derived Induced Pluripotent Stem Cells as a Model to Study Frontotemporal Dementia PathologiesSemantic proximity Read ↗

Astrocytic Reactivity Triggered by defective Mitophagy Activates NF-kB Signaling and Causes Neurotoxicity in Frontotemporal Dementia Type 3Semantic proximity Read ↗

Generation and validation of an iPSC line HMSCATi009-A from a patient with frontotemporal dementia.Semantic proximity Read ↗

An iPSC-Derived Neuron Model of CLN3 Disease Facilitates Small Molecule Phenotypic ScreeningSemantic proximity Read ↗

Frontotemporal dementia patient-derived iPSC neurons show cell pathological hallmarks and evidence for synaptic dysfunction and DNA damageSemantic proximity Read ↗

Generation of a human induced pluripotent stem cell (iPSC) line (IBMS-iPSC-070-02) from a patient with neurodegeneration with brain iron accumulation (NBIA) having compound heterozygous mutations in PANK2 gene.Semantic proximity Read ↗

Astrocytic reactivity triggered by defective autophagy and metabolic failure causes neurotoxicity in frontotemporal dementia type 3.Semantic proximity Read ↗

Glutamate-Glutamine Homeostasis is Perturbed in Neurons and Astrocytes derived from Patient iPSC Models of Frontotemporal DementiaSemantic proximity Read ↗

The role of CHMP2BIntron5 in autophagy and frontotemporal dementia.Semantic proximity Read ↗

Mutations in EPG5 are associated with a wide spectrum of neurodevelopmental and neurodegenerative disordersSemantic proximity Read ↗

Generation and validation of a human iPSC-derived TDP-43 knockout model for ALS disease modelingSemantic proximity Read ↗

Genetics of neurodegeneration with brain iron accumulation.Semantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.