MYBPC1 mutations impair skeletal muscle function in zebrafish models of arthrogryposis

View Full Paper

Ask AI

Bookmark

Population

Zebrafish embryos

Comparison

Antisense morpholino knockdown of mybpc1… vs Overexpression of wild-type RNA

Design

Preclinical

Authors

Discussion

Zebrafish model supports dominant-negative MYBPC1 effects in arthrogryposis; leaves open human therapeutic translation.

Population

Zebrafish embryos

Intervention

Antisense morpholino knockdown of mybpc1; injection of mybpc1 mRNAs containing human W236R and Y856H MYBPC1 mutations

Comparator

Overexpression of wild-type RNA (for mutation experiments)

Outcome

Embryonic motor activity, survival, body curvature, cardiac edema, myofibril organization, and sarcomere numberssurrogate

Demonstrates a critical role for mybpc1 in slow skeletal muscle development and establishes zebrafish as a model for human distal arthrogryposis.

Ha et al. (2013) studied this question.

View Full Paper

Ask AI

Bookmark