Nederlandse Vereniging voor Klinische Chemie en Laboratoriumgeneeskunde
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Project 277 Desmosine as a biomarker for elastin degradation in COPD and IPF
Onderzoekslijn Desmosine als biomarker voor elastine degradatie
Omschrijving Although chronic obstructive pulmonary disease (COPD) and idiopathic pulmonary fibrosis (IPF) seem to be opposite entities from a clinical perspective, a classic animal model suggests common initial pathogenic steps in both lung diseases. Intratracheal administration of cadmium chloride causes fibrosis in hamster’s lungs, while combining this toxin with a lathyrogen, an inhibitor of lysyl oxidase (LO), leads to emphysema. LO does not only play a key-role in the perinatal development of durable elastin fibers by crosslinking the precursor tropoelastin, but also, by the same mechanism, in the repair of damaged elastin fibers. During this crosslinking process the amino acids desmosine and isodesmosine (DES) arise, which are unique to mature elastin. Without adequate LO-crosslinking, the newly synthesized tropoelastin-proteins are extremely vulnerable for degradation by proteases. The rate of systemic mature elastin degradation is most reliably quantified by LC-MS/MS based blood DES assay. The COPD-Biomarker-Qualification-Consortium appreciated that DES has great potential as a COPD-biomarker since DES is central to pathogenesis. However, data regarding the value and concentrations of DES in IPF patients is currently lacking.
Projectleider Dr. J.M.W.(Jody) van den Ouweland
Instituut Canisius-Wilhelmina Ziekenhuis Nijmegen
Trefwoorden Idiopathic pulmonary fibrosis COPD desmosine elastin
Status Lopend
Periode 1-2015 - 1-2025
Financiering 1e Geldstroom - Intern