What is the clinical evidence from this study?

Study design: Other. Population: Dilated cardiomyopathy and mitochondrial dysfunction. Intervention: Cardiac-specific Lonp1 knock-out vs. Cre-control mice.

What type of study is this?

This is a Cohort Study study (also classified as: Experimental Study).

October 9, 2025Circulation Research

Abstract Fri131: Mitochondrial Lonp1 Regulates Mitochondrial Inner Membrane Quality Control In Cardiac Functions

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Key result

Cardiac-specific Lonp1 knockout causes lethal dilated cardiomyopathy with 0% survival beyond 21 days.

Population

Cre-control and cardiac-specific Lonp1 knock-out (Lonp1 cKO) mice (n=24 for weight assessments, n=6 for TEM)

Comparison

Cardiac-specific Lonp1 knock-out vs Cre-control mice

Design

Preclinical

Follow-up

up to 21 days

Authors

SMSakthijothi MuthuWest Virginia University ZTZinnia TranWest Virginia University PSPratikshya ShresthaWest Virginia University

Discussion

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Implication

Lonp1 is essential for inner mitochondrial membrane integrity and cardiac function, with its deletion leading to fatal dilated cardiomyopathy in mice.

Key Points

Lonp1 knock-out mice displayed severe dilated cardiomyopathy, demonstrating its crucial role in cardiac health.
Mitochondrial evaluations showed significant reductions in inner membrane proteins and cristae integrity in Lonp1 cKO mice.
Histological analyses indicated notable ventricular dilation and increased cardiac fibrosis associated with Lonp1 deficiency.
Cre control mice evidenced normal mitochondrial and cardiac function, contrasting sharply with Lonp1 cKO findings.

Structured PICO

Population

Cardiac-specific Lonp1 knock-out (Lonp1 cKO) and Cre-control mice evaluated for cardiac structure, mitochondrial integrity, and energy production.

Intervention

Cardiac-specific Lonp1 knock-out

Comparator

Cre-control mice

Outcome

Cardiac structure, mitochondrial and cristae integrity, and mitochondrial functionsurrogate

Lonp1 is essential for inner mitochondrial membrane integrity and cardiac function, with its deletion leading to fatal dilated cardiomyopathy in mice.

Cite This Study

Muthu et al. (2025) studied Dilated cardiomyopathy and mitochondrial dysfunction. Cardiac-specific Lonp1 knock-out vs. Cre-control mice was evaluated. Cardiac-specific Lonp1 knock-out in mice resulted in severe ventricular dilation, disrupted inner mitochondrial membrane, and no survival beyond 21 days.

synapsesocial.com/papers/68e80eb363e2e2f707877d83 https://doi.org/10.1161/res.137.suppl_1.fri131

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Implication

Lonp1 is essential for inner mitochondrial membrane integrity and cardiac function, with its deletion leading to fatal dilated cardiomyopathy in mice.

Abstract Fri131: Mitochondrial Lonp1 Regulates Mitochondrial Inner Membrane Quality Control In Cardiac Functions

Discussion

Member takes

Implication

Key Points

Structured PICO

Cite This Study

Abstract Fri131: Mitochondrial Lonp1 Regulates Mitochondrial Inner Membrane Quality Control In Cardiac Functions

Discussion

Member takes

Implication

Key Points

Structured PICO

Cite This Study

Also Consider

Also Consider