Obscurin

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{{STRUCTURE_2yz8| PDB=2yz8 | SIZE=400| SCENE= |right|CAPTION=Human obscurin of 32th IG-like domain residues 3184-3273 [[2yz8]] }}
 
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<StructureSection load='2yz8' size='350' side='right' caption='Human obscurin of 32th IG-like domain residues 3184-3273 (PDB entry [[2yz8]])' scene=''>
== Function ==
== Function ==
'''Obscurin''' (Obs) or '''KIAA1556''' is a giant sarcomeric signalling protein expressed in striated muscles. Other giant proteins in the family include titin and nebulin. It is a multidomain protein containing adhesion modules and signaling domains. These domains include: 68 Ig domains, 2 fibronectin domains, 1 calcium/calmodulin-binding domain, 1 RhoGEF domain with PH domain and 2 serine/thereonine kinase domains.
'''Obscurin''' (Obs) or '''KIAA1556''' is a giant sarcomeric signalling protein expressed in striated muscles. Other giant proteins in the family include titin and nebulin. It is a multidomain protein containing adhesion modules and signaling domains. These domains include: 68 Ig domains, 2 fibronectin domains, 1 calcium/calmodulin-binding domain, 1 RhoGEF domain with PH domain and 2 serine/thereonine kinase domains.
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== Disease ==
== Disease ==
Mutations of Obs are found in hypertrophy cardiomyopathy<ref>PMID:17716621</ref>.
Mutations of Obs are found in hypertrophy cardiomyopathy<ref>PMID:17716621</ref>.
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</StructureSection>
==3D structures of obscurin==
==3D structures of obscurin==

Revision as of 10:00, 28 August 2017

Human obscurin of 32th IG-like domain residues 3184-3273 (PDB entry 2yz8)

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3D structures of obscurin

28-August-2017

References

  1. Kontrogianni-Konstantopoulos A, Catino DH, Strong JC, Sutter S, Borisov AB, Pumplin DW, Russell MW, Bloch RJ. Obscurin modulates the assembly and organization of sarcomeres and the sarcoplasmic reticulum. FASEB J. 2006 Oct;20(12):2102-11. PMID:17012262 doi:10.1096/fj.06-5761com
  2. Arimura T, Matsumoto Y, Okazaki O, Hayashi T, Takahashi M, Inagaki N, Hinohara K, Ashizawa N, Yano K, Kimura A. Structural analysis of obscurin gene in hypertrophic cardiomyopathy. Biochem Biophys Res Commun. 2007 Oct 19;362(2):281-7. Epub 2007 Aug 13. PMID:17716621 doi:10.1016/j.bbrc.2007.07.183

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