Obscurin: Difference between revisions
Michal Harel (talk | contribs) No edit summary |
Michal Harel (talk | contribs) No edit summary |
||
| Line 1: | Line 1: | ||
{{STRUCTURE_2yz8| PDB=2yz8 | SIZE=400| SCENE= |right|CAPTION=Human obscurin of 32th IG-like domain residues 3184-3273 [[2yz8]] }} | {{STRUCTURE_2yz8| PDB=2yz8 | SIZE=400| SCENE= |right|CAPTION=Human obscurin of 32th IG-like domain residues 3184-3273 [[2yz8]] }} | ||
== 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. | ||
Obscurin binds small ankyrin 1 which is part of the sarcoplasmic reticulum membrane. It associates with the contractile apparatus via binding to titin and sarcomeric myosin. Obscurin is assumed to play a key role in modulating the organization and assembly of sarcomeric reticulum and the myofibril<ref>PMID:17012262</ref>. | Obscurin binds small ankyrin 1 which is part of the sarcoplasmic reticulum membrane. It associates with the contractile apparatus via binding to titin and sarcomeric myosin. Obscurin is assumed to play a key role in modulating the organization and assembly of sarcomeric reticulum and the myofibril<ref>PMID:17012262</ref>. | ||
For more details see [[Group:MUZIC:Obscurin]]. | For more details see [[Group:MUZIC:Obscurin]]. | ||
== Disease == | |||
Mutations of Obs are found in hypertrophy cardiomyopathy<ref>PMID:17716621</ref>. | |||
==3D structures of obscurin== | ==3D structures of obscurin== | ||