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February 24, 2016Skeletal MuscleOpen Access

Attenuated Ca2+ release in a mouse model of limb girdle muscular dystrophy 2A

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Key result

Calpain-3 knockout mice, a model for limb girdle muscular dystrophy 2A, exhibited a significant 14% reduction in peak Ca2+ fluxes elicited by single action potentials compared to wild-type mice.

Population

Single fibers isolated from hind limb muscles of young wild-type and calpain-3 knockout mice

Comparison

Ablation of CAPN3 (calpain-3 knockout) vs Wild-type (WT) mice

Design

Preclinical

Authors

MDMarino DiFrancoIKIrina KramerovaJVJulio L. Vergara

Discussion

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Member takes

Overview

Indicates early Ca2+ release defect in LGMD2A model; leaves open contribution to CaMKII changes and human relevance.

Structured PICO

P
Population
Male wild-type and calpain-3 knockout (C3KO) mice aged 9.5 to 16.5 weeks, studied as a model for limb girdle muscular dystrophy 2A.
E
Exposure
Ablation of CAPN3 (calpain-3 knockout)
C
Comparator
Wild-type (WT) mice
O
Outcome
Ca2+ fluxes/release elicited by single action potentials and tetanic stimulationsurrogate

Main Result

Absolute Event Rate: 229% vs 265%

p-value: p=<0.05

Decreased Ca2+ release is an early defect in calpainopathy (LGMD2A) and may contribute to the observed reduction of CaMKII activation in C3KO mice.

Limitations

  • The single compartment model used does not incorporate explicit equations for the voltage dependence of Ca2+ release.

Cite This Study

DiFranco et al. (2016) studied Limb girdle muscular dystrophy 2A (LGMD2A) mouse model. CAPN3 ablation (C3KO) vs. Wild-type (WT) mice was evaluated on Peak Ca2+ fluxes elicited by single action potentials (μM/ms) (p=<0.05). Calpain-3 knockout mice, a model for limb girdle muscular dystrophy 2A, exhibited a significant 14% reduction in peak Ca2+ fluxes elicited by single action potentials compared to wild-type mice.

synapsesocial.com/papers/6ab08261f94dfcbfc8f06c6fhttps://doi.org/10.1186/s13395-016-0081-y
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Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Loss of calpain 3 dysregulates store-operated calcium entry and its exercise response in mice2024 · 2 citations
  2. 2Loss of Calpain 3 dysregulates store‐operated calcium entry and its exercise response in mice2024 · 3 citations
  3. 3CaMKIIβ Signaling drives expression of metabolic and stress response genes in skeletal muscle, and its loss contributes to the LGMDR1 phenotype2026
  4. 4Mitochondrial dysfunction and consequences in calpain-3-deficient muscle2020 · 22 citations
  5. 5Calpain 3 is important for muscle regeneration: Evidence from patients with limb girdle muscular dystrophies2012 · 63 citations