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

Muscular dystrophy-associated lamin variants disrupt cellular organization through a nucleolar-ribosomal axis.

Science advances - 28 Aug 2026

Ding Xiangyi, Kumari Sweta, F Gregory Ellen, Starr Daniel A, Luxton G W Gant

Abstract excerpt

Emery-Dreifuss muscular dystrophy (EDMD) arises from mutations in nuclear lamins or emerin. Current pathological models emphasize defective nuclear mechanics and transcriptional regulation, yet these mechanisms cannot explain how lamina defects propagate across the cell to produce the complex pathology of laminopathies. Here, we reveal an emerging pathway linking nuclear lamina dysfunction to cytoplasmic...

Read the complete abstract on PubMed

Topics

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

Share to Caenorhabditis elegans Proteins
Share to Cell Nucleolus

Methodology Data sources Editorial policy Privacy Delete account & data About Contact Helena Bioinformatics

Share to Cytoplasm

Share to Endoplasmic Reticulum

Share to Lamins

Share to Muscular Dystrophy, Emery-Dreifuss

Share to Nuclear Lamina

Share to Ribosomes

Source

Open in PubMed Open full text

Identifiers

PMID: 42647638
DOI: 10.1126/sciadv.aec9028
PMCID: PMC13510739

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 24 Oct 2026.

Related research

Semantic proximity does not establish scientific evidence. Graph snapshot: 2026-09-29.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Muscular dystrophy-associated lamin variants disrupt cellular organization through a nucleolar-ribosomal axis.PMID 42647638Read ↗

Muscular dystrophy-associated lamin variants disrupt cellular organization through a nucleolar-ribosomal axis controlling cytoplasmic macromolecular crowdingSemantic proximity Read ↗

Impaired mechanical response of an EDMD mutation leads to motility phenotypes that are repaired by loss of prenylation.Semantic proximity Read ↗

A laminopathic mutation disrupting lamin filament assembly causes disease-like phenotypes in Caenorhabditis elegans.Semantic proximity Read ↗

Laminopathic mutations interfere with the assembly, localization, and dynamics of nuclear lamins.Semantic proximity Read ↗

Myopathic lamin mutations cause reductive stress and activate the nrf2/keap-1 pathway.Semantic proximity Read ↗

The Fall of the Armor: Lamin Dysregulation and a Wide Network of Laminopathies.Semantic proximity Read ↗

Lamin A/C Assembly Defects in LMNA-Congenital Muscular Dystrophy Is Responsible for the Increased Severity of the Disease Compared with Emery-Dreifuss Muscular Dystrophy.Semantic proximity Read ↗

Caenorhabditis elegans as a model system for studying the nuclear lamina and laminopathic diseases.Semantic proximity Read ↗

Changes in Chromatin Organisation and Mechanotransduction Mediated by Mutant Lamins in Laminopathies.Semantic proximity Read ↗

Diverse lamin-dependent mechanisms interact to control chromatin dynamics. Focus on laminopathies.Semantic proximity Read ↗

Genome-wide analysis links emerin to neuromuscular junction activity in Caenorhabditis elegans.Semantic proximity Read ↗

Rewiring Fibroblast–Muscle Axis Drives Progressive Pathology in Bethlem MyopathySemantic proximity Read ↗

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