LMNA

1 article(s) in the watch · Constitutional genetics

LMNA encodes nuclear-envelope lamins A/C. Its variants cause a broad spectrum of laminopathies.

InheritanceAutosomal dominant

Clinical spectrumDilated cardiomyopathy with conduction disease, muscular dystrophies, lipodystrophy, progeria

ManagementClose cardiac surveillance; defibrillator often indicated (high arrhythmic risk).

Curated publications

High-Risk Cardiomyopathy Genotypes and Arrhythmic Risk: LMNA, FLNC, RBM20, PLN and Desmosomal Genes in the ESC 2023 Era

Hereditary cardiomyopathies (HCM, DCM) with high risk of ventricular arrhythmia and sudden death

How is a LMNA-related condition inherited?+

Autosomal dominant

What is the clinical spectrum associated with LMNA?+

Dilated cardiomyopathy with conduction disease, muscular dystrophies, lipodystrophy, progeria

What is the management associated with LMNA?+

Close cardiac surveillance; defibrillator often indicated (high arrhythmic risk).

How many Geno'X publications cover the LMNA gene?+

1 publication(s) on LMNA have been selected, summarised and scored by Geno'X on a public grading grid (domains: Constitutional genetics).