Discover Community Create You

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

Article

Proteasome impairment in neural cells derived from HMSN-P patient iPSCs.

Molecular brain - 15 Feb 2017

Murakami Nagahisa, Imamura Keiko, Izumi Yuishin, Egawa Naohiro, Tsukita Kayoko, Enami Takako, Yamamoto Takuya, Kawarai Toshitaka, Kaji Ryuji, Inoue Haruhisa

Abstract excerpt

Hereditary motor and sensory neuropathy with proximal dominant involvement (HMSN-P) is caused by a heterozygous mutation (P285L) in Tropomyosin-receptor kinase Fused Gene (TFG), histopathologically characterized by progressive spinal motor neuron loss with TFG cytosolic aggregates. Although the TFG protein, found as a type of fusion oncoprotein, is known to facilitate vesicle transport from endoplasmic reticulum...

Read the complete abstract on PubMed

Topics

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

Share to Clone Cells
Share to Hereditary Sensory and Motor Neuropathy

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Induced Pluripotent Stem Cells

Share to Motor Neurons

Share to Neurons

Share to Proteasome Endopeptidase Complex

Share to Proteins

Share to Spinal Cord

Source

Open in PubMed Open full text

Identifiers

PMID: 28196470
DOI: 10.1186/s13041-017-0286-y
PMCID: PMC5310050

Genes

TFG

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 5 Sept 2026. Manifest publication-graph-cohort-2026-09-05.1.

Source receipt: pubmed, retrieved 5 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Proteasome impairment in neural cells derived from HMSN-P patient iPSCs.PMID 28196470Read ↗

Neuromuscular dysfunction in patient-derived FUS <sup>R244RR</sup> -ALS iPSC model via axonal downregulation of neuromuscular junction proteinsSemantic proximity Read ↗

Evidence of TRK-Fused Gene (TFG1) function in the ubiquitin-proteasome system.Semantic proximity Read ↗

Proteomic analysis of human iPSC derived neural stem cells and motor neurons identifies proteasome structural alterations.Semantic proximity Read ↗

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

Establishment of In Vitro FUS-Associated Familial Amyotrophic Lateral Sclerosis Model Using Human Induced Pluripotent Stem CellsSemantic proximity Read ↗

TFG p.G269V Mutation Disrupts Motor Neuron Function in iPSC-Derived Models via Wnt Signaling Dysregulation.Semantic proximity Read ↗

Induced pluripotent stem cell-derived motor neurons of CMT type 2 patients reveal progressive mitochondrial dysfunction.Semantic proximity Read ↗

Integrated profiling of iPSC-derived motor neurons carrying C9orf72, FUS, TARDBP, or SOD1 mutations.Semantic proximity Read ↗

Defective autophagy and increased apoptosis contribute toward the pathogenesis of FKRP-associated muscular dystrophies.Semantic proximity Read ↗

Patient iPSC-derived neural stem cells exhibit phenotypes in concordance with the clinical severity of mucopolysaccharidosis I.Semantic proximity Read ↗

Frontotemporal dementia-associated N279K tau mutant disrupts subcellular vesicle trafficking and induces cellular stress in iPSC-derived neural stem cellsSemantic proximity Read ↗

Rapid degeneration of iPSC-derived motor neurons lacking Gdap1 engages a mitochondrial-sustained innate immune responseSemantic proximity Read ↗

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