Titin-based stiffening of muscle fibers in Ehlers-Danlos Syndrome | Zendy

Coen A. C. Ottenheijm | Zendy; Nicol C. Voermans | Zendy; Bryan D. Hudson | Zendy; Thomas C. Irving | Zendy; Ger J.M. Stienen | Zendy; Baziel G.M. van Engelen | Zendy; Henk Granzier | Zendy

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Titin-based stiffening of muscle fibers in Ehlers-Danlos Syndrome

Author(s) -

Coen A. C. Ottenheijm,

Nicol C. Voermans,

Bryan D. Hudson,

Thomas C. Irving,

Ger J.M. Stienen,

Baziel G.M. van Engelen,

Henk Granzier

Publication year - 2012

Publication title -

journal of applied physiology

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 1.253

H-Index - 229

eISSN - 8750-7587

pISSN - 1522-1601

DOI - 10.1152/japplphysiol.01166.2011

Subject(s) - titin , obscurin , sarcomere , myofilament , extracellular matrix , extracellular , chemistry , medicine , biophysics , endocrinology , biology , myocyte , biochemistry , actin

tenascin-X (TNX) is an extracellular matrix glycoprotein whose absence leads to Ehlers-Danlos Syndrome (EDS). TNX-deficient EDS patients present with joint hypermobility and muscle weakness attributable to increased compliance of the extracellular matrix. We hypothesized that in response to the increased compliance of the extracellular matrix in TNX-deficient EDS patients, intracellular adaptations take place in the elastic properties of the giant muscle protein titin.

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