Cardiomyopathy

Cardiomyopathy encompasses a spectrum of heart muscle disorders characterized by structural and functional dysfunction. The major subtypes — hypertrophic (HCM), dilated (DCM), restrictive (RCM), and arrhythmogenic right ventricular cardiomyopathy (ARVC) — frequently arise from mutations in sarcomeric genes. Key pathological hallmarks include ventricular wall thickening, impaired diastolic function, and elevated risk of life-threatening arrhythmias, making these conditions critical targets for preclinical mouse model research.

7+
Validated Models
40
Studies Completed
10
IND Studies Completed

Advantages

Our Advantages

Industry-leading capabilities for Coronary Heart Disease (CHD)

Advanced Cardiac Imaging

Longitudinal echocardiographic monitoring of cardiac remodeling and dysfunction.

Specialized HCM Analysis

Proprietary PV loop data and analytical capabilities in HCM study.

Comprehensive Drug Development

Extensive model resources and pharmacodynamic expertise, including small molecules, AAV-based therapies, gene correction therapeutics.

Model Library

CHD Models

SR-BI Mutation

Case Studies

Case Studies & Validation Data

Explore our studies demonstrating the efficacy of various therapeutic interventions.

Mybpc3 KO

Echocardiographic Characterization of Mybpc3 -/- (KO) Mice.

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(A) Representative B-mode images at 2 weeks. (B) Longitudinal cardiac parameters (0-40 weeks) in WT vs. Mybpc3 KO mice. Top row: Wall thickness and mass (IVS;d, IVS;s, LVPW;d, LVMass). Bottom row: Chamber volume and function (LVESV, LVEDV, EF%, SV). KO mice show progressive hypertrophy, dilation, and declining function.

Mybpc3 KO Hearts Show Progressive Pathology

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Gross morphology, H&E, and Masson's trichrome stains at 2, 3, and 5 weeks. Compared to WT, Mybpc3 KOhearts show progressive ventricular dilation, myofiber disarray, tissue damage, and replacement fibrosis, indicating severe pathological remodeling.

Mybpc3 KO Mice Exhibit Cardiac Hypertrophy and Remodeling.

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(A) Gross heart morphology shows enlargement. (B) HW/BW ratio is significantly increased (****p<0.0001) in both sexes. (C) H&E staining reveals cellular disarray. (D) Masson’s trichrome highlights fibrosis.

Myh6 R404Q

Echocardiographic Characterization of Myh6 R404Q/+ Mice.

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(A) B-mode images at 4 months show left ventricular chamber dilation in Myh6 R404Q*/+ vs. WT. (B) Longitudinal data (5-25 wks) plot wall thickness (LVPW;s, IVS;d), dimensions (LVID;d/s), volume (LVESV, LVEDV), and function (EF, CO), demonstrating pathological remodeling.

*Note: Myh6 R404Q corresponds to MYH7 R403Q.

PV Loop of Myh6 R404Q/+ Mice.

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(A) Bar graphs: Mutants show ↑Ved, ↑Ves (dilation), ↑Tau (impaired relaxation) vs. Control. (B) Box plots: ↓ESPVR (reduced contractility), altered EDPVR (stiffness). (C) PV loops: Mutant loops are right-shifted and enlarged, confirming severe systolic dysfunction (****p<0.0001).

Myh6 R404Q+/- Hearts Show Fibrotic Pathology.

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(A) H&E staining reveals tissue disruption (yellow arrows) in mutant hearts. (B) Masson’s trichrome staining highlights extensive collagen deposition (blue, yellow arrow) in mutants, especially females. (C) Fibrosis quantification confirms a significant increase (*p<0.05) in mutant mice (~2.5% in males, ~5% in females) versus WT (~1% males, ~0.5% females).

Mavacamten improves the HCM phenotype of the Myh6.pR404C mice

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Echocardiographic parameters (IVS;d, LVID;s, LVPW;d, LVEDV, LVESV, EF, FS, LV Mass, RWT) were tracked from baseline to 8 weeks in three groups: G1 (WT+Vehicle), G2 (Myh6-p.R404Q+Vehicle), and G3 (Myh6-p.R404Q+Mavacamten, 2 mg/kg). Data points show mean±SEM.

Models

All Product Lines

Browse our comprehensive catalog of over 30,000 mouse models covering various gene families, signaling pathways, and disease models. Use advanced filters to find the perfect model for your research.