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
  • Molecular Sequence Data
  • Body Patterning
  • Animals
  • Myofibrils/metabolism*
  • Myofibrils/ultrastructure
  • Somites/metabolism
  • Somites/ultrastructure
  • Sequence Alignment
  • Conserved Sequence
  • Heart/embryology
  • Microscopy, Electron, Transmission
  • Muscle Proteins/chemistry
  • Muscle Proteins/genetics
  • Muscle Proteins/metabolism*
  • Phylogeny
  • Gene Expression Regulation, Developmental
  • Guanine Nucleotide Exchange Factors/chemistry
  • Guanine Nucleotide Exchange Factors/genetics
  • Guanine Nucleotide Exchange Factors/metabolism*
  • Embryo, Nonmammalian/embryology
  • Embryo, Nonmammalian/metabolism
  • Humans
  • Myosin-Light-Chain Kinase/metabolism
  • Zebrafish Proteins/chemistry
  • Zebrafish Proteins/genetics
  • Zebrafish Proteins/metabolism*
  • Amino Acid Sequence
  • Myocardium/metabolism
  • Zebrafish/embryology*
  • Zebrafish/genetics
  • Zebrafish/metabolism*
(all 31)
PubMed
16779859 Full text @ Dev. Dyn.
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
Figures
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Expression
Phenotype
Mutations / Transgenics
No data available
Human Disease / Model
No data available
Sequence Targeting Reagents
Target Reagent Reagent Type
obscnbMO1-obscnbMRPHLNO
obscnbMO2-obscnbMRPHLNO
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Fish
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Antibodies
Orthology
No data available
Engineered Foreign Genes
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Mapping
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