PUBLICATION
Obscurin is required for the lateral alignment of striated myofibrils in zebrafish
- Authors
- Raeker, M.O., Su, F., Geisler, S.B., Borisov, A.B., Kontrogianni-Konstantopoulos, A., Lyons, S.E., and Russell, M.W.
- ID
- ZDB-PUB-060623-5
- Date
- 2006
- Source
- Developmental Dynamics : an official publication of the American Association of Anatomists 235(8): 2018-2029 (Journal)
- Registered Authors
- Lyons, Susan, Su, Fengyun
- Keywords
- obscurin, zebrafish, myofibrillogenesis, somite, sarcomere, heart, development
- MeSH Terms
-
- Somites/metabolism
- Somites/ultrastructure
- Myocardium/metabolism
- Myofibrils/metabolism*
- Myofibrils/ultrastructure
- Phylogeny
- Sequence Alignment
- Gene Expression Regulation, Developmental
- Muscle Proteins/chemistry
- Muscle Proteins/genetics
- Muscle Proteins/metabolism*
- Conserved Sequence
- Zebrafish Proteins/chemistry
- Zebrafish Proteins/genetics
- Zebrafish Proteins/metabolism*
- Molecular Sequence Data
- Microscopy, Electron, Transmission
- Body Patterning
- Embryo, Nonmammalian/embryology
- Embryo, Nonmammalian/metabolism
- Humans
- Myosin-Light-Chain Kinase/metabolism
- Animals
- Heart/embryology
- Amino Acid Sequence
- Zebrafish/embryology*
- Zebrafish/genetics
- Zebrafish/metabolism*
- Guanine Nucleotide Exchange Factors/chemistry
- Guanine Nucleotide Exchange Factors/genetics
- Guanine Nucleotide Exchange Factors/metabolism*
- PubMed
- 16779859 Full text @ Dev. Dyn.
Citation
Raeker, M.O., Su, F., Geisler, S.B., Borisov, A.B., Kontrogianni-Konstantopoulos, A., Lyons, S.E., and Russell, M.W. (2006) Obscurin is required for the lateral alignment of striated myofibrils in zebrafish. Developmental Dynamics : an official publication of the American Association of Anatomists. 235(8):2018-2029.
Abstract
Obscurin/obscurin-MLCK is a giant sarcomere-associated protein with multiple isoforms whose interactions with titin and small ankyrin-1 suggest that it has an important role in myofibril assembly, structural support, and the sarcomeric alignment of the sarcoplasmic reticulum. In this study, we characterized the zebrafish orthologue of obscurin and examined its role in striated myofibril assembly. Zebrafish obscurin was expressed in the somites and central nervous system by 24 hours post-fertilization (hpf) and in the heart by 48 hpf. Depletion of obscurin using two independent morpholino antisense oligonucleotides resulted in diminished numbers and marked disarray of skeletal myofibrils, impaired lateral alignment of adjacent myofibrils, disorganization of the sarcoplasmic reticulum, somite segmentation defects, and abnormalities of cardiac structure and function. This is the first demonstration that obscurin is required for vertebrate cardiac and skeletal muscle development. The diminished capacity to generate and organize new myofibrils in response to obscurin depletion suggests that it may have a vital role in the causation of or adaptation to cardiac and skeletal myopathies.
Genes / Markers
Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Orthology
Engineered Foreign Genes
Mapping