SCIENTIFIC RESEARCH:

December 2007
LATEST Publication Paper on Molecular Pathogenisis of LAM
published in American Journal of Respiratory, Cell and Molecular Biology

Lymphangioleiomyomatosis (LAM) is a rare progressive cystic lung disease affecting young women. The pivotal observation that LAM occurs both spontaneously and as part of the tuberous sclerosis complex (TSC) led to the hypothesis that these disorders share common genetic and pathogenetic mechanisms. In this review we describe the evolution of our understanding of the molecular and cellular basis of LAM and TSC, beginning with the discovery of the TSC1 and TSC2 genes and the demonstration of their involvement in sporadic (non-TSC) LAM. This was followed by rapid delineation of the signaling pathways in Drosophila melanogaster with confirmation in mice and humans. This knowledge served as the foundation for novel therapeutic approaches that are currently being used in human clinical trials. > more

Collaborative Programme of Scientific Investigation into LAM

As a result of three years of work by The New Zealand LAM Trust, the team of New Zealand and Australian researchers (pictured right) are working against the clock as part of an international collaborative effort to uncover answers to the causes of this rare and cruel disease.

Dr Lyn Moir began her work at The University of Pennsylvania in the lab of Dr Vera Krymskaya in June 2007. Dr Moir will return to The University of Sydney in 2008, and it is hoped that funds will be raised to enable her to continue her cell signalling work there.

A presentation abstract on the work completed in Philadelphia will be made at the American Thoracic Society's annual meeting in Toronto in 2008 and at the Australasian Thoracic meeting in Melbourne in May 2008.

Lymphangioleiomyomatosis
by Francis McCormack, MD

Lymphangioleiomyomatosis (LAM) is a rare, progressive, cystic lung disease that occurs almost exclusively in females, usually between menarche and menopause. The hallmarks of LAM are diffuse infiltration of the pulmonary parenchyma with atypical smooth muscle cells, airflow obstruction, pneumothorax and chylothorax, and progressive respiratory failure... > more

Australian Scientific Investigation

In June 2004 the prestigious UK Journal of Pathology published a Paper from Professor Mervyn J Merrilees, principal investigator,University of Auckland in conjunction with Professor Judith L. Black, University of Sydney. The paper is titled "Matrix Proteoglycans and remodelling of interstial lung tissue in Lymphangioleiomyomatosis (LAM)".

This Paper can be seen on line in Wylie InterScience (www.interscience.wiley.com) and is published in J Pathol 2004; no 203: 653-660.

Currently, NZ LAM Trust funded LAM science investigation continues at the University of Auckland.

Professor Merrilees and his team's recent studies (UK Journal of Path) have shown that LAM is characterised not only by the growth of defective smooth muscle cells, but also by significant amounts of interstitial tissue that contain normal fibroblasts and large amounts of space occupying matrix proteoglycans.

Of particular interest is the finding that areas rich in proteoglycans are depleted in elastic fibres which are essential to the proper functioning of the lung during breathing.

Prof Merrilees and the team have been working with the drug rapamycin which has been found to be a very effective inhibiter of cell migration.

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