Titin

Titin, also known as connectin, is a protein that in humans is encoded by the TTN gene. It is the largest known protein and plays a key role in muscle contraction.

Structure

Titin is a giant protein, greater than 1 µm in length, that functions as a molecular spring which is responsible for the passive elasticity of muscle. It is composed of 244 individually folded protein domains connected by unstructured peptide sequences. These domains are organized into a series of repeating structural motifs.

Function

Titin is a critical component of the sarcomere, contributing to its assembly and structure, and its elasticity. It connects the Z line to the M line in the sarcomere. The protein functions as a molecular spring which is responsible for the passive elasticity of muscle.

Clinical significance

Mutations in this gene are associated with idiopathic dilated cardiomyopathy (IDC) and familial hypertrophic cardiomyopathy (FHC).

See also

• Sarcomere
• Muscle contraction
• Cardiomyopathy

References

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External links

• TTN gene at NCBI

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