Tenascin-X deficiency and Ehlers-Danlos syndrome: a case report and review of the literature.

O'Connell, M; Burrows, N P; van Vlijmen-Willems, M J J; Clark, S M; Schalkwijk, J · Br J Dermatol · 2010

case_report · Level V

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Abstract

Tenascin-X is a large extracellular matrix glycoprotein that is widely distributed within connective tissues and is associated with an autosomal recessive type of Ehlers-Danlos syndrome (EDS). Tenascin-X represents the first EDS susceptibility gene that does not code for a fibrillar collagen or collagen-processing enzyme. We describe a paediatric case of tenascin-X deficiency and review the literature.

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