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September 4, 2018European Journal of Heart FailureOpen Access

Lamin Missense Mutations—The Spectrum of Phenotype Variability is Increasing

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Key result

LMNA missense variants expressing less mutant lamin show a more favorable clinical trajectory.

Why the study?

The clinical outcomes and phenotype variability of LMNA missense mutations in cardiolaminopathies are poorly understood, complicating risk stratification and management.

Design

Editorial discussing clinical and genetic aspects of LMNA missense mutations

Authors

GCGabriella CapturZBZofia T. BilińskaEAEloisa Arbustini

Discussion

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Overview

May inform LMNA cardiomyopathy prognosis; leaves open validation of expression ratios for personalized risk stratification.

PICO

P
Population
Cardiolaminopathies
E
Exposure / Comparator
LMNA missense mutations

The editorial highlights the increasing recognition of phenotypic variability in LMNA missense mutations and emphasizes the need for functional studies and personalized management to predict clinical expression and outcomes.

Limitations

  • Our ability to predict whether and when an LMNA mutation carrier will eventually manifest with heart disease remains suboptimal.

Cite This Study

Captur et al. (2018) conducted an editorial in Cardiolaminopathies. LMNA missense mutations was evaluated. LMNA missense mutations exhibit wide phenotypic variability, with variants like p.Arg216Cys expressing less mutant lamin (30:70 mut/wt) and showing a more favorable clinical trajectory.

synapsesocial.com/papers/6aaf2b6ca7e31fa2e934f82fhttps://doi.org/10.1002/ejhf.1290
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