PUBLICATION

Zebrafish ambra1a and ambra1b Knockdown Impairs Skeletal Muscle Development

Authors
Skobo, T., Benato, F., Grumati, P., Meneghetti, G., Cianfanelli, V., Castagnaro, S., Chrisam, M., Di Bartolomeo, S., Bonaldo, P., Cecconi, F., Valle, L.D.
ID
ZDB-PUB-140613-2
Date
2014
Source
PLoS One   9: e99210 (Journal)
Registered Authors
Benato, Francesca, Skobo, Tatjana
Keywords
Embryos, Skeletal muscles, Zebrafish, Muscle fibers, Autophagic cell death, Muscle proteins, Somites, Muscle differentiation
MeSH Terms
  • Adaptor Proteins, Signal Transducing/deficiency
  • Adaptor Proteins, Signal Transducing/genetics
  • Adaptor Proteins, Signal Transducing/metabolism*
  • Animals
  • Birefringence
  • Cell Proliferation
  • Embryo, Nonmammalian/abnormalities
  • Embryo, Nonmammalian/metabolism
  • Embryo, Nonmammalian/pathology
  • Gene Expression Regulation, Developmental/drug effects
  • Gene Knockdown Techniques*
  • Mice
  • Morpholinos/pharmacology
  • Movement
  • Muscle Development/genetics*
  • Muscle Fibers, Skeletal/drug effects
  • Muscle Fibers, Skeletal/pathology
  • Muscle Fibers, Skeletal/ultrastructure
  • Muscle, Skeletal/abnormalities
  • Muscle, Skeletal/drug effects
  • Muscle, Skeletal/embryology*
  • Muscle, Skeletal/metabolism
  • MyoD Protein/metabolism
  • Myosins/metabolism
  • PAX7 Transcription Factor/metabolism
  • Zebrafish/embryology*
  • Zebrafish/genetics*
  • Zebrafish Proteins/deficiency
  • Zebrafish Proteins/genetics
  • Zebrafish Proteins/metabolism*
PubMed
24922546 Full text @ PLoS One
Abstract

The essential role of autophagy in muscle homeostasis has been clearly demonstrated by phenotype analysis of mice with muscle-specific inactivation of genes encoding autophagy-related proteins. Ambra1 is a key component of the Beclin 1 complex and, in zebrafish, it is encoded by two paralogous genes, ambra1a and ambra1b, both required for normal embryogenesis and larval development. In this study we focused on the function of Ambra1, a positive regulator of the autophagic process, during skeletal muscle development by means of morpholino (MO)-mediated knockdown and compared the phenotype of zebrafish Ambra1-depleted embryos with that of Ambra1gt/gt mouse embryos. Morphological analysis of zebrafish morphant embryos revealed that silencing of ambra1 impairs locomotor activity and muscle development, as well as myoD1 expression. Skeletal muscles in ATG-morphant embryos displayed severe histopathological changes and contained only small areas of organized myofibrils that were widely dispersed throughout the cell. Double knockdown of ambra1a and ambra1b resulted in a more severe phenotype whereas defects were much less evident in splice-morphants. The morphants phenotypes were effectively rescued by co-injection with human AMBRA1 mRNA. Together, these results indicate that ambra1a and ambra1b are required for the correct development and morphogenesis of skeletal muscle.

Genes / Markers
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Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Antibodies
Orthology
Engineered Foreign Genes
Mapping