Table 2.

Electrophysiology in hiPSC-CM models of dACM. For each mutation studied in hiPSC-CMs for which data are available, we summarized findings related to cardiomyocyte electrical activity, including molecular and functional data for individual ion channels and parameters related to excitation, conduction, and synchrony in multicellular preparations. Mutations are given in p-dot (p.) nomenclature and specified as T (truncating) or M (missense). Symbols and colors are described below the information presented in the table and comparisons are represented as mutant cell lines relative to control cell lines under the same conditions as presented by the data published in the primary sources, for which references are provided.

									Molecular Data									Single Channel Currents										Excitation, Conduction, & Synchrony
Gene	Mutation	SCN5A/Nav1.5	GJA/Cx43	SK3/KCa2.3	SK4/KCa3.1	KCNJ11/Kir6.2	KCNQ1/Kv7.1	KCNJ2/Kir2.1	KCNH2/Kv11.1	SLC8A1/NCX1	INa	ICaL	INCX	Ito	IK	Ikr	Iks	ISK	ISK4	IKATP	AP probability	Resting potential	AP upstroke velocity	AP upstroke heterogeneity	AP amplitude	AP duration	AP frequency	Conduction velocity	Arrhythmic events	Refs.
PKP2	p.G828LfsX835	▽									▼											■	▼		▼	■				[28,68]
	p.A324fsX355		▼																				▼							[58]
	p.Y119MfsX141 + p.K859R	■■□									▼												▼			■				[84]
	p.D109AfsX119 *		▼																							▲				[85]
	p.D109AfsX119 *		▼																							▲
DSG2	p.G638R †	▽		▽	▲△	▽					▼	■	▼	▼		▲	■	▼	▲	▼		■	▼		▼	■	▲		▲	[89,182]
	p.D787MfsX807	△	□				△	□		□													■	▲		▼		■		[59]
	p.R119X																											▼	▲	[90]
DSP	p.R451G		▼																									■		[91]
	p.H1684R										▼	▼		▲							▼				▼	▼				[92]
DSC2	p.R132C						△		△		▼				▲							■	■		■	▼	▲		▲	[88,185,186]
Heterozygous mutation (not shaded)											▼ decreased relative to control
Compound heterozygous mutation (shaded red)											■ no change relative to control
Homozygous mutation (shaded blue)											▲ increased relative to control
											▲■▼ membrane protein (immunofluoresence)
* Artificially introduced mutation (not patient derived)											▲■▼ total protein abundance (Western blot)
† Assumed heterozygous, although not specified											△□▽ total mRNA abundance (qPCR/RNA-seq)