Fibrillin controls TGF-β activation
V Kaartinen, D Warburton - Nature genetics, 2003 - nature.com
Nature genetics, 2003•nature.com
Secreted transforming growth factor-βs (TGF-βs) are rendered biologically inactive by
binding proteins that also target and concentrate them to the extracellular matrix. Specific,
but still poorly understood, activation is required for disassembly of the extracellular matrix–
bound protein complex to liberate the mature growth factor and to obtain a correct biological
effect. A new study shows that fibrillin-1, the protein defective in Marfan syndrome, has a
biologically important role in controlling TGF-β activation in the lung.
binding proteins that also target and concentrate them to the extracellular matrix. Specific,
but still poorly understood, activation is required for disassembly of the extracellular matrix–
bound protein complex to liberate the mature growth factor and to obtain a correct biological
effect. A new study shows that fibrillin-1, the protein defective in Marfan syndrome, has a
biologically important role in controlling TGF-β activation in the lung.
Secreted transforming growth factor-βs (TGF-βs) are rendered biologically inactive by binding proteins that also target and concentrate them to the extracellular matrix. Specific, but still poorly understood, activation is required for disassembly of the extracellular matrix–bound protein complex to liberate the mature growth factor and to obtain a correct biological effect. A new study shows that fibrillin-1, the protein defective in Marfan syndrome, has a biologically important role in controlling TGF-β activation in the lung.
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