. 2019 Aug 7;20(16):3844. doi: 10.3390/ijms20163844

Table 1.

Effects of TRPV2 blockade on muscular dystrophy and cardiomyopathy.

TRPV2 Blocker Gene, Agents	Animal Models/Cells for Human Disease	Strain	Background of Animals	Evaluation Index (Efficacy)	Reports
TRPV2-NT Transgenic or adenovirus	* DCM mice	4C30	sialyltransferase transgenic	cardiac function↑, heart weight↓,	[9]
	* DCM mice	4C30	sialyltransferase transgenic	fibrosis↓, survival↑ * CK↓,* ANP↓, * cTn-I↓ in serum	[9]
	DCM mice	* TNNT2 *ΔK210	cTn-T mutant knock-in	cardiac function↑	[17]
	DCM hamsters	J2N-k	δ-sarcoglycan defect	cardiac function↑	[9]
	* DOX induced CM	BL6	cardiotoxicity	cardiac function↑, *ROS production↓	[9]
*** DN-TRPV2 Transgenic or adenovirus**	* MD mice	mdx	dystrophin defect	fibrosis↓, serum CK↓, Ca²⁺ influx↓, recovery of muscle strength↑	[18,19]
*** DN-TRPV2 Transgenic or adenovirus**	MD hamsters	BIO14.6	δ-sarcoglycan defect	fibrosis↓, serum CK↓, Ca²⁺ influx↓	[18]
Functional antibody	DCM mice	4C30	sialyltransferase transgenic	cardiac function↑	[20,21,22]
	DCM hamsters	J2N-k	δ-sarcoglycan defect	cardiac function↑, serum CK↓	[20,21,22]
	MD cardiomyocytes	mdx	dystrophin defect	stretch induced Ca²⁺ influx↓ in isolated cardiomyocytes	[23,24,25]
	TAC mice	BL6	hemodynamic stress	cardiac function↑	[22]
Inhibitors *tranilast, lumin**	DCM mice	4C30	sialyltransferase transgenic	cardiac function↑, fibrosis↓	[26,27]
	DCM hamsters	J2N-k	δ-sarcoglycan defect	cardiac function↑, fibrosis↓	[9,28]
	MD cardiomyocytes	mdx	dystrophin defect	stretch induced Ca²⁺ influx↓	[23,24,25]
	MD mice	* DKO	utrophin/dystrophin defect	cardiac function↑	Figure 3
Gene silencing	MD cardiomyocytes	mdx	dystrophin defect	Ca²⁺ influx↓	[23]
antisense DNA, * siRNA	MD myotubes	BIO14.6	δ-sarcoglycan defect	Ca²⁺ influx↓	[8]
Gene deletion	* TAC mice	BL6/129S	hemodynamic stress	hypertrophy↓, cardiac function→	[12]
(Knockout)	* MI mice	BL6/129S	ischemia stress	cardiac function↑	[13,14]

* DCM, dilated cardiomyopathy; * MD, muscular dystrophy; * CK, creatine phosphokinase; * ANP, atrial natriuretic peptide; * cTn-I, cardiac troponin-I; * TNNT2, cardiac troponin-T; * Δ210K, 210Lys deletion; * DOX, doxorubicin; * ROS, reactive oxygen species; * DN, dominant negative; * lumin (NK-4), 4,4′-[3-[2-[1-ethyl-4(1H)-quinolinylidene] ethylidene]-1-propene-1,3-diyl]bis(1-ethylquinolinium) diiodide; * DKO, double knockout mouse; * siRNA, small interfering RNA; * TAC, transverse aortic constriction; * MI, myocardial infarction; ↑, increased or improved; ↓, decreased or ameliorated; →, no change.