Obscurin: Difference between revisions

Revision as of 12:49, 15 September 2020

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 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^[1].

For more details see Group:MUZIC:Obscurin.

Disease

Mutations of Obs are found in hypertrophy cardiomyopathy^[2].

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

Drag the structure with the mouse to rotate

3D structures of obscurin3D structures of obscurin

15-September-2020

ReferencesReferences

↑ 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
↑ 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

Proteopedia Page Contributors and Editors (what is this?)Proteopedia Page Contributors and Editors (what is this?)

Michal Harel, Alexander Berchansky

[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

[1]

[2]

@@ Line 17: / Line 17: @@
 **[[2e7b]] - hObs 6th Ig domain residues 8-97 – human - NMR<BR />
+**[[2eo1]] - hObs Ig domain residues 69-163 – NMR<br />
 **[[2dm7]] - hObs 14th Ig domain residues 1157-1251 – NMR<br />
 **[[2gqh]] - hObs 15th Ig domain residues 1253-1346 – NMR<br />
@@ Line 38: / Line 39: @@
 *Obscurin-like protein 1
+**[[5fm5]] - hObsL 3rd Ig domain residues 251-339 + myomesin-1<br />
 **[[2e6q]] - hObsL Ig domain residues 615-713 – NMR<BR />
 **[[2e6p]] - hObsL Ig domain residues 714-804 – NMR<BR />
-**[[3knb]], [[2wp3]], [[2wwm]], [[4c4k]] – hObs N terminal residues 1-105 + titin M10 domain <br />
+**[[2lvc]] - hObsL 6th Ig domain residues 805-892 – NMR<br />
-**[[4uow]] - hObs residues 26829-26925+ titin M10 domain<br />
+**[[2lu7]] - hObsL 11th Ig domain residues 1277-1357 – NMR<br />
-**[[2wwk]] – hObs N terminal residues 1-105 (mutant) + titin M10 domain<br />
+**[[3knb]], [[2wp3]], [[2wwm]], [[4c4k]] – hObsL N terminal residues 1-105 + titin M10 domain <br />
+**[[2wwk]] – hObsL N terminal residues (mutant) + titin M10 domain<br />
+**[[4uow]] - hObsL residues 26829-26925 + titin M10 domain<br />
 }}
 == References ==
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 [[Category:Topic Page]]