Nebulin: Difference between revisions
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Michal Harel (talk | contribs) New page: <StructureSection load='1neb' size='350' side='right' caption='NMR structure of human nebulin SH3 domain (PDB entry 1neb)' scene=''> '''Nebulin''' is an actin-binding protein whic... |
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<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<ref>PMID:21115852</ref>. | |||
For more details see [[Group:MUZIC:Nebulin]]. | |||
== Disease == | |||
Mutations in nebulin were identified in patients with nemaline myopathy<ref>PMID:11257471</ref>. | |||
==3D structures of nebulin== | ==3D structures of nebulin== | ||
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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 /> | ||
== References == | |||
<references/> | |||
Revision as of 10:53, 5 May 2016
<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 05-May-2016
1neb – hNebulin SH3 domain residues 918-977 – human - NMR
1ark – hNebulin SH3 domain residues 6610-6669 - NMR
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
