Telethonin

Function

Telethonin is found in muscle cells and binds titin Z1-Z2 domains. It is essential to sarcomer assembly^[1]. It is called titin cap protein. See also Group:MUZIC:Telethonin.

Disease

Mutations in telethonin lead to heart maladptation to biomechanical stress^[2].

Crystal structure of telethonin (dark green) in complex with titin domains z1z2 dimer (grey and light green) and sulfate (PDB code 1ya5)

Drag the structure with the mouse to rotate

3D Structures of Telethonin3D Structures of Telethonin

2f8v, 1ya5 - telethonin+ hTTN residues 1-196 - human

ReferencesReferences

↑ Faulkner G, Lanfranchi G, Valle G. Telethonin and other new proteins of the Z-disc of skeletal muscle. IUBMB Life. 2001 May;51(5):275-82. PMID:11699871 doi:10.1080/152165401317190761
↑ Knoll R, Linke WA, Zou P, Miocic S, Kostin S, Buyandelger B, Ku CH, Neef S, Bug M, Schafer K, Knoll G, Felkin LE, Wessels J, Toischer K, Hagn F, Kessler H, Didie M, Quentin T, Maier LS, Teucher N, Unsold B, Schmidt A, Birks EJ, Gunkel S, Lang P, Granzier H, Zimmermann WH, Field LJ, Faulkner G, Dobbelstein M, Barton PJ, Sattler M, Wilmanns M, Chien KR. Telethonin deficiency is associated with maladaptation to biomechanical stress in the mammalian heart. Circ Res. 2011 Sep 16;109(7):758-69. doi: 10.1161/CIRCRESAHA.111.245787. Epub, 2011 Jul 28. PMID:21799151 doi:http://dx.doi.org/10.1161/CIRCRESAHA.111.245787

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Alexander Berchansky, Michal Harel, Joel L. Sussman

[1] Faulkner G, Lanfranchi G, Valle G. Telethonin and other new proteins of the Z-disc of skeletal muscle. IUBMB Life. 2001 May;51(5):275-82. PMID:11699871 doi:10.1080/152165401317190761

[2] Knoll R, Linke WA, Zou P, Miocic S, Kostin S, Buyandelger B, Ku CH, Neef S, Bug M, Schafer K, Knoll G, Felkin LE, Wessels J, Toischer K, Hagn F, Kessler H, Didie M, Quentin T, Maier LS, Teucher N, Unsold B, Schmidt A, Birks EJ, Gunkel S, Lang P, Granzier H, Zimmermann WH, Field LJ, Faulkner G, Dobbelstein M, Barton PJ, Sattler M, Wilmanns M, Chien KR. Telethonin deficiency is associated with maladaptation to biomechanical stress in the mammalian heart. Circ Res. 2011 Sep 16;109(7):758-69. doi: 10.1161/CIRCRESAHA.111.245787. Epub, 2011 Jul 28. PMID:21799151 doi:http://dx.doi.org/10.1161/CIRCRESAHA.111.245787

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