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The Journal of Cell Biology
Article
License: CC BY NC SA
Data sources: UnpayWall
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PubMed Central
Other literature type . 2014
Data sources: PubMed Central
The Journal of Cell Biology
Article . 2014 . Peer-reviewed
Data sources: Crossref
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The nebulin repeat protein Lasp regulates I-band architecture and filament spacing in myofibrils

Authors: Isabelle Fernandes; Frieder Schöck;

The nebulin repeat protein Lasp regulates I-band architecture and filament spacing in myofibrils

Abstract

Mutations in nebulin, a giant muscle protein with 185 actin-binding nebulin repeats, are the major cause of nemaline myopathy in humans. Nebulin sets actin thin filament length in sarcomeres, potentially by stabilizing thin filaments in the I-band, where nebulin and thin filaments coalign. However, the precise role of nebulin in setting thin filament length and its other functions in regulating power output are unknown. Here, we show that Lasp, the only member of the nebulin family in Drosophila melanogaster, acts at two distinct sites in the sarcomere and controls thin filament length with just two nebulin repeats. We found that Lasp localizes to the Z-disc edges to control I-band architecture and also localizes at the A-band, where it interacts with both actin and myosin to set proper filament spacing. Furthermore, introducing a single amino acid change into the two nebulin repeats of Lasp demonstrated different roles for each domain and established Lasp as a suitable system for studying nebulin repeat function.

Related Organizations
Keywords

Sarcomeres, Microfilament Proteins, Muscle Proteins, Myosins, Myopathies, Nemaline, Actins, Protein Structure, Tertiary, Drosophila melanogaster, Myofibrils, Animals, Drosophila Proteins, Actinin, Connectin, Muscle, Skeletal, Research Articles, Cytoskeleton

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    This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
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    influence
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citations
This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
42
Top 10%
Top 10%
Top 10%
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