The NH2 terminus of titin spans the Z-disc: its interaction with a novel 19-kD ligand (T-cap) is required for sarcomeric integrity

J Cell Biol. 1998 Nov 16;143(4):1013-27. doi: 10.1083/jcb.143.4.1013.

Abstract

Titin is a giant elastic protein in vertebrate striated muscles with an unprecedented molecular mass of 3-4 megadaltons. Single molecules of titin extend from the Z-line to the M-line. Here, we define the molecular layout of titin within the Z-line; the most NH2-terminal 30 kD of titin is located at the periphery of the Z-line at the border of the adjacent sarcomere, whereas the subsequent 60 kD of titin spans the entire width of the Z-line. In vitro binding studies reveal that mammalian titins have at least four potential binding sites for alpha-actinin within their Z-line spanning region. Titin filaments may specify Z-line width and internal structure by varying the length of their NH2-terminal overlap and number of alpha-actinin binding sites that serve to cross-link the titin and thin filaments. Furthermore, we demonstrate that the NH2-terminal titin Ig repeats Z1 and Z2 in the periphery of the Z-line bind to a novel 19-kD protein, referred to as titin-cap. Using dominant-negative approaches in cardiac myocytes, both the titin Z1-Z2 domains and titin-cap are shown to be required for the structural integrity of sarcomeres, suggesting that their interaction is critical in titin filament-regulated sarcomeric assembly.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Actinin / chemistry
  • Actinin / metabolism
  • Amino Acid Sequence
  • Animals
  • Cells, Cultured
  • Chick Embryo
  • Connectin
  • Gene Expression / physiology
  • Membrane Proteins / chemistry
  • Membrane Proteins / genetics
  • Membrane Proteins / metabolism
  • Microscopy, Immunoelectron
  • Molecular Sequence Data
  • Muscle Fibers, Skeletal / cytology
  • Muscle Fibers, Skeletal / metabolism
  • Muscle Fibers, Skeletal / ultrastructure
  • Muscle Proteins / chemistry*
  • Muscle Proteins / genetics
  • Muscle Proteins / metabolism*
  • Muscle, Skeletal / chemistry
  • Muscle, Skeletal / cytology
  • Muscle, Skeletal / metabolism
  • Myocardium / chemistry
  • Myocardium / cytology
  • Myocardium / metabolism
  • Myofibrils / chemistry
  • Myofibrils / metabolism
  • Myofibrils / ultrastructure
  • Protein Kinases / chemistry*
  • Protein Kinases / genetics
  • Protein Kinases / metabolism*
  • Protein Structure, Tertiary
  • Sarcomeres / chemistry*
  • Sarcomeres / metabolism*
  • Sarcomeres / ultrastructure
  • Transcription, Genetic / physiology

Substances

  • Connectin
  • Membrane Proteins
  • Muscle Proteins
  • Actinin
  • Protein Kinases