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(Chest. 2002;121:56S-61S.)
© 2002 American College of Chest Physicians

Genetics and Gene Expression in Lymphangioleiomyomatosis*

Giles F. Filley Lecture

Gustavo Pacheco-Rodriguez, PhD; Arnold S. Kristof, MD; Linda A. Stevens; Yi Zhang, PhD; Denise Crooks, PhD and Joel Moss, MD, PhD

* From the Pulmonary-Critical Care Medicine Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD.

Correspondence to: Joel Moss, MD, PhD, Chief, Pulmonary-Critical Care Medicine Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, 10 Center Dr, Bldg 10, Room 6 D05, MSC 1590, Bethesda, MD 20892-1590; e-mail: mossj{at}nhlbi.nih.gov

Lymphangioleiomyomatosis (LAM) is a disease of unknown etiology that is characterized by the proliferation of abnormal smooth muscle cells (LAM cells) in the lung, which leads to cystic parenchymal destruction and progressive respiratory failure. Recent evidence suggests that the proliferative and invasive nature of LAM cells may be due, in part, to somatic mutations in the TSC2 gene, which has been implicated in the pathogenesis of tuberous sclerosis complex. Here, we describe the clinical and molecular characteristics of LAM, as well as the efforts now under way to understand the genetic and biochemical factors that lead to progressive pulmonary destruction and, ultimately, to lung transplantation or death.

Key Words: lymphangioleiomyomatosis • tuberous sclerosis complex




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