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

Multiple lines of evidence for disruption of nuclear lamina and nucleoporins in FUS amyotrophic lateral sclerosis.

Brain : a journal of neurology - 4 Nov 2024

Okada Kensuke, Ito Daisuke, Morimoto Satoru, Kato Chris, Oguma Yuki, Warita Hitoshi, Suzuki Naoki, Aoki Masashi, Kuramoto Junko, Kobayashi Reona, Shinozaki Munehisa, Ikawa Masahito, Nakahara Jin, Takahashi Shinichi, Nishimoto Yoshinori, Shibata Shinsuke, Okano Hideyuki

Abstract excerpt

Advanced pathological and genetic approaches have revealed that mutations in fused in sarcoma/translated in liposarcoma (FUS/TLS), which is pivotal for DNA repair, alternative splicing, translation and RNA transport, cause familial amyotrophic lateral sclerosis (ALS). The generation of suitable animal models for ALS is essential for understanding its pathogenesis and developing therapies. Therefore, we used...

Read the complete abstract on PubMed

Topics

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

Share to Amyotrophic Lateral Sclerosis
Share to Induced Pluripotent Stem Cells

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Mice, Transgenic

Share to Motor Neurons

Share to Nuclear Lamina

Share to Nuclear Pore Complex Proteins

Share to RNA-Binding Protein FUS

Share to Spinal Cord

Source

Open in PubMed Open full text

Identifiers

PMID: 39312484
DOI: 10.1093/brain/awae224
PMCID: PMC11684083

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Multiple lines of evidence for disruption of nuclear lamina and nucleoporins in FUS amyotrophic lateral sclerosis.PMID 39312484Read ↗

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

Impaired DNA damage response signaling by FUS-NLS mutations leads to neurodegeneration and FUS aggregate formation.Semantic proximity Read ↗

FUS unveiled in mitochondrial DNA repair and targeted ligase-1 expression rescues repair-defects in FUS-linked motor neuron disease.Semantic proximity Read ↗

Mislocated FUS is sufficient for gain-of-toxic-function amyotrophic lateral sclerosis phenotypes in mice.Semantic proximity Read ↗

Activation of polo-like kinase 1 correlates with selective motor neuron vulnerability in familial ALSSemantic proximity Read ↗

Amyotrophic Lateral Sclerosis, FUS and Protein Synthesis Defects.Semantic proximity Read ↗

The fused in sarcoma protein forms cytoplasmic aggregates in motor neurons derived from integration-free induced pluripotent stem cells generated from a patient with familial amyotrophic lateral sclerosis carrying the FUS-P525L mutation.Semantic proximity Read ↗

FUS Unveiled in Mitochondrial DNA Repair and Targeted Ligase-1 Expression Rescues Repair-Defects in FUS-Linked NeurodegenerationSemantic proximity Read ↗

Directly converted patient-specific induced neurons mirror the neuropathology of FUS with disrupted nuclear localization in amyotrophic lateral sclerosis.Semantic proximity Read ↗

Autosomal dominant inheritance of rapidly progressive amyotrophic lateral sclerosis due to a truncation mutation in the fused in sarcoma (FUS) gene.Semantic proximity Read ↗

FUS/TLS deficiency causes behavioral and pathological abnormalities distinct from amyotrophic lateral sclerosis.Semantic proximity Read ↗

Pathology of three ALS patients with FUS variants, including one likely benign Q23L variant lacking FUS inclusions.Semantic proximity Read ↗

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