Telomere length is a determinant of emphysema susceptibility.

ABSTRACT:

Rationale

Germline mutations in the enzyme telomerase cause telomere shortening, and have their most common clinical manifestation in age-related lung disease that manifests as idiopathic pulmonary fibrosis. Short telomeres are also a unique heritable trait that is acquired with age.

Objectives

We sought to understand the mechanisms by which telomerase deficiency contributes to lung disease.

Methods

We studied telomerase null mice with short telomeres.

Measurements and main results

Although they have no baseline histologic defects, when mice with short telomeres are exposed to chronic cigarette smoke, in contrast with controls, they develop emphysematous air space enlargement. The emphysema susceptibility did not depend on circulating cell genotype, because mic

SUBMITTER: Alder JK 

PROVIDER: S-EPMC3208661 | biostudies-literature | 2011 Oct

REPOSITORIES: biostudies-literature