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Nat Genet. 2004 Feb;36(2):178-82. Epub 2004 Jan 25.

Elastic fiber homeostasis requires lysyl oxidase-like 1 protein.

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1
Berman-Gund Laboratory for the Study of Retinal Degenerations, Harvard Medical School and Massachusetts Eye & Ear Infirmary, Boston, Massachusetts 02114, USA.

Abstract

Elastic fibers are components of the extracellular matrix and confer resilience. Once laid down, they are thought to remain stable, except in the uterine tract where cycles of active remodeling occur. Loss of elastic fibers underlies connective tissue aging and important diseases including emphysema. Failure to maintain elastic fibers is explained by a theory of antielastase-elastase imbalance, but little is known about the role of renewal. Here we show that mice lacking the protein lysyl oxidase-like 1 (LOXL1) do not deposit normal elastic fibers in the uterine tract post partum and develop pelvic organ prolapse, enlarged airspaces of the lung, loose skin and vascular abnormalities with concomitant tropoelastin accumulation. Distinct from the prototypic lysyl oxidase (LOX), LOXL1 localizes specifically to sites of elastogenesis and interacts with fibulin-5. Thus elastin polymer deposition is a crucial aspect of elastic fiber maintenance and is dependent on LOXL1, which serves both as a cross-linking enzyme and an element of the scaffold to ensure spatially defined deposition of elastin.

PMID:
14745449
DOI:
10.1038/ng1297
[Indexed for MEDLINE]
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