期刊
FEBS LETTERS
卷 584, 期 14, 页码 2990-2994出版社
WILEY
DOI: 10.1016/j.febslet.2010.06.011
关键词
Arterial tortuosity; Glucose transporter; Ascorbate; Elastin; Collagen
资金
- University of Pennsylvania School of Dental Medicine
Arterial Tortuosity Syndrome (ATS) is a heritable disease characterized by twisting and lengthening of the major arteries, hypermobility of the joints, and laxity of skin. ATS is caused by mutations in SLC2A10, encoding Glucose Transporter 10 (GLUT10). The current model of ATS holds that loss of GLUT10 at the nuclear periphery induces a glucose-dependent increase in Transforming Growth Factor-beta (TGF beta) that stimulates vessel wall cell proliferation. Instead, we propose that GLUT10 transports ascorbate, a cofactor for collagen and elastin hydroxylases, into the secretory pathway. In ATS, loss of GLUT10 results in defective collagen and/or elastin. TGF beta activation represents a secondary response to a defective extracellular matrix. (C) 2010 Federation of European Biochemical Societies. Published by Elsevier B.V. All rights reserved.
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