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Article

Extracellular matrix turnover and disease severity in Anderson-Fabry disease.

Journal of inherited metabolic disease - 1 Feb 2007

Shah J S, Hughes D A, Tayebjee M H, MacFadyen R J, Mehta A B, Elliott P M

Abstract excerpt

BACKGROUND: Anderson-Fabry Disease (AFD) is an inherited metabolic disease associated with premature death secondary to cardiovascular and renal disease. Patients with AFD develop progressive left ventricular (LV) remodelling and heart failure. We hypothesized that altered extracellular matrix (ECM) turnover contributes to the pathophysiology of cardiac disease in AFD. METHODS AND RESULTS: Twenty-nine consecutive...

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Identifiers

PMID: 17160618
DOI: 10.1007/s10545-006-0360-6

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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