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Article

Early mechanical dysfunction of the diaphragm in the muscular dystrophy with myositis (Ttnmdm) model.

American journal of physiology. Cell physiology - 1 Nov 2008

Lopez Michael A, Pardo Patricia S, Cox Gregory A, Boriek Aladin M

Abstract excerpt

A complex rearrangement mutation in the mouse titin gene leads to an in-frame 83-amino acid deletion in the N2A region of titin. Autosomal recessive inheritance of the titin muscular dystrophy with myositis (Ttn(mdm/mdm)) mutation leads to a severe early-onset muscular dystrophy and premature death. We hypothesized that the N2A deletion would negatively impact the force-generating capacity and passive mechanical...

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Source

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Identifiers

PMID: 18753318
DOI: 10.1152/ajpcell.16.2008
PMCID: PMC2584999

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.

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