What is the clinical evidence from this study?

Study design: Other. Population: Congenital myopathy. Intervention: Cypher knockout vs. Wild-type. Primary outcome: Phenotype of Cypher knockout mice.

What question did this study set out to answer?

Investigate the role of Cypher protein in congenital myopathy and its function in muscle maintenance.

November 5, 2001The Journal of Cell BiologyOpen Access

Ablation of Cypher, a PDZ-LIM domain Z-line protein, causes a severe form of congenital myopathy

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Key result

Cypher knockout mice display a severe form of congenital myopathy and die postnatally from functional failure in multiple striated muscles due to failure in Z-line maintenance.

Population

Cypher knockout mice

Design

Preclinical

Authors

QZQiang ZhouJishou University PCPo‐Hsien ChuChang Gung Memorial Hospital CHChenqun HuangSan Diego State University

Discussion

Member takes

Implication

Hypothesis-generating for Cypher-related congenital myopathy; leaves open Z-line maintenance as a therapeutic target in humans.

Key Points

Investigate the role of Cypher protein in congenital myopathy and its function in muscle maintenance.
Utilized Cypher knockout mice to observe effects of ablation on muscle function and morphology.
Examined striated muscle for sarcomerogenesis and Z-line assembly.
Conducted in vitro studies to assess domain interactions with Z-line components.
Cypher knockout mice exhibit severe congenital myopathy and die shortly after birth.
Cypher is essential for maintaining the Z-line during muscle function, but not for initial assembly or sarcomerogenesis.
In vitro studies confirm Cypher's role as a linker for cytoskeletal stability.

Structured PICO

Population

Cypher knockout mice

Intervention

Ablation of the Cypher gene

Outcome

Development of congenital myopathy and postnatal survival

Cypher is essential for maintaining Z-line structure during muscle contraction, and its absence leads to severe congenital myopathy and postnatal death.

Cite This Study

Zhou et al. (2001) studied Congenital myopathy. Cypher knockout vs. Wild-type was evaluated on Phenotype of Cypher knockout mice. Cypher knockout mice display a severe form of congenital myopathy and die postnatally from functional failure in multiple striated muscles due to failure in Z-line maintenance.

synapsesocial.com/papers/6a07b3e744ff8ad339f69a69 https://doi.org/10.1083/jcb.200107092

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Ablation of Cypher, a PDZ-LIM domain Z-line protein, causes a severe form of congenital myopathy

Discussion

Member takes

Implication

Key Points

Structured PICO

Cite This Study

Ablation of Cypher, a PDZ-LIM domain Z-line protein, causes a severe form of congenital myopathy

Discussion

Member takes

Implication

Key Points

Structured PICO

Cite This Study

Also Consider

Also Consider