reSeArcH Article
Extended Data Fig. 1 | LMNA-mutant iPSC-CMs can recapitulate
arrhythmic phenotype of patients with LMNA-related DCM.
a, Schematic pedigree of the family carrying the mutation in LMNA.
Patients (III-1, III-3, III-9, III-15 and III-17) and healthy individuals
(IV-1 and IV-2) recruited for this study are numbered. Circles represent
female family members and squares represent males. The ‘+’ and ‘−’
signs underneath family members indicate the presence or absence of
the mutation in LMNA, respectively. b, Schematic view of 348-349insG
frameshift mutation in LMNA. c, Genotyping of fibroblasts derived from
patients and healthy controls. MT, mutant. d, Clinical features of patients
and healthy individuals. e, Electrocardiogram of patients III-1 and
III-3 and a healthy individual (IV-1). The electrocardiogram data were
measured once per individual. f, g, Electrophysiological measurements
of spontaneous action potentials in mutant iPSC-CMs (III-15 and III-17)
recorded by patch clamp in current-clamp mode. The experiments were
repeated three times independently with similar results.