Serum endostatin levels are associated with diffusion capacity and with tuberous sclerosis- associated lymphangioleiomyomatosis.

Abstract

Endostatin is a naturally occurring collagen fragment with anti-angiogenic properties. We investigated the association between serum endostatin levels and DLCO in a cohort of patients with lymphangioleiomyomatosis (LAM). Associations of endostatin levels to clinical features of LAM were explored using logistic regression models. Endostatin levels were associated with DLCO and were higher in subjects with TSC-associated LAM compared to sporadic LAM. These data suggest that endostatin could be a predictive biomarker of decline in DLCO and that germline mutational inactivation of the TSC1 or TSC2 gene is associated with higher endostatin levels. These findings could offer novel insights into the pathogenesis of LAM.

• Authors: Anthony M Lamattina, Sergio Poli, Pranav Kidambi, Shefali Bagwe, Andrew Courtwright, Pierce H Louis, Shikshya Shrestha, Benjamin Stump, Hilary J Goldberg, Elizabeth A Thiele, Ivan Rosas, Elizabeth P Henske, Souheil El-Chemaly
• Journal: Orphanet journal of rare diseases (Orphanet J Rare Dis) Vol. 14 Issue 1 Pg. 72 (03 29 2019) ISSN: 1750-1172 [Electronic] England
• PMID: 30922357 (Publication Type: Letter, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)

Chemical References

• Biomarkers
• Endostatins
• TSC1 protein, human
• TSC2 protein, human
• Tuberous Sclerosis Complex 1 Protein
• Tuberous Sclerosis Complex 2 Protein

Topics

• Adult
• Biomarkers (blood)
• Cohort Studies
• Endostatins (blood, genetics)
• Female
• Gene Silencing
• Germ-Line Mutation
• Humans
• Lymphangioleiomyomatosis (blood, complications, genetics, physiopathology)
• Middle Aged
• Tuberous Sclerosis (complications)
• Tuberous Sclerosis Complex 1 Protein (genetics)
• Tuberous Sclerosis Complex 2 Protein (genetics)