Nebulin: Difference between revisions
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[[1neb]] – hNebulin SH3 domain residues 918-977 – human - NMR <br /> | [[1neb]] – hNebulin SH3 domain residues 918-977 – human - NMR <br /> | ||
[[1ark]] – hNebulin SH3 domain residues 6610-6669 - NMR <br /> | [[1ark]] – hNebulin SH3 domain residues 6610-6669 - NMR <br /> | ||
[[7qin]] – mNebulin in actomyosin complex – mouse – Cryo EM<br /> | |||
[[7qim]] – mNebulin + actin – Cryo EM<br /> | |||
== References == | == References == | ||
<references/> | <references/> | ||
[[Category:Topic Page]] | [[Category:Topic Page]] | ||
Revision as of 10:08, 11 July 2023
<StructureSection load='1neb' size='350' side='right' caption='NMR structure of human nebulin SH3 domain (PDB entry 1neb)' scene=>
Function
Nebulin is an actin-binding protein which can bind upto 200 actin molecules. Nebulin length is proportional to the length of the thin filament. It may function as a regulator of thin filament length during sarcomere assembly[1].
For more details see Group:MUZIC:Nebulin.
Disease
Mutations in nebulin were identified in patients with nemaline myopathy[2].
3D structures of nebulin
Updated on 11-July-2023
1neb – hNebulin SH3 domain residues 918-977 – human - NMR
1ark – hNebulin SH3 domain residues 6610-6669 - NMR
7qin – mNebulin in actomyosin complex – mouse – Cryo EM
7qim – mNebulin + actin – Cryo EM
References
- ↑ Labeit S, Ottenheijm CA, Granzier H. Nebulin, a major player in muscle health and disease. FASEB J. 2011 Mar;25(3):822-9. doi: 10.1096/fj.10-157412. Epub 2010 Nov 29. PMID:21115852 doi:https://dx.doi.org/10.1096/fj.10-157412
- ↑ Gurgel-Giannetti J, Reed U, Bang ML, Pelin K, Donner K, Marie SK, Carvalho M, Fireman MA, Zanoteli E, Oliveira AS, Zatz M, Wallgren-Pettersson C, Labeit S, Vainzof M. Nebulin expression in patients with nemaline myopathy. Neuromuscul Disord. 2001 Mar;11(2):154-62. PMID:11257471