TABLE 2.

Monosubunit mutation and their locus and consequence.

Mutant Subunit	Mutant Locus	Consequence
Kir 6.1	Smooth muscle	Hypertension/hypotension
	Lymphatic smooth muscle	Lymphatic contractile dysfunction lymphatic smooth muscle hyperpolarization
	Skeletal muscle	Reduced limb strength, skeletal muscle atrophy, autophagy, and myofibers connective tissue replacement
	S422L	Shortened repolarization in ventricular tissue
		Increase atrial fibrillation susceptibility
Kir 6.2	Pancreatic islet cell	Neonatal diabetes mellitus, maturity-onset diabetes of the young 13, type 2 diabetes mellitus, and even persistent hyperinsulinemic hypoglycemia of infancy
	rs5215 G/G	Vasodilation augment and shear stress reduction
	Hypertension mouse model	Heart failure and death, myocardial incommensurate remodeling
	p.V59M	Intellectual disability
SUR1	Pancreas	Neonatal diabetes
	V187D	Higher insulin secretion in hypoglycemia and make KATP channel acting pharmaceuticals out of action
	p.H1401Tfs	Clinical heterogeneity congenital hyperinsulinemia
	Unstated	Increased channel activity in MgATP/MgADP, reduced the KATP channel surface expression
SUR2A	Fs1524 and A1513T	Severely dilated hearts with impaired systolic function and arrhythmia
SUR2B	R659C	Heart disease and early repolarization syndrome
	C24S and C1455S	Prevent the detrimental effects of sulfhydration and NaHS-induced tyrosine nitration