期刊
FEBS OPEN BIO
卷 5, 期 -, 页码 257-263出版社
WILEY
DOI: 10.1016/j.fob.2015.03.009
关键词
ATP-binding cassette sub-family C member 6 (ABCC6); Bone morphogenetic protein (BMP); BMP signaling; Mouse; Liver; Kidney
资金
- National Institutes of Health [R01 HL81397, R01 HL112839, R01 NS79353, P1 HL30568]
Mutations in ABCC6 (ATP-binding cassette, subfamily C, member 6), an orphan transporter expressed in the liver, are the cause of pseudoxanthoma elasticum. Since ABCC6 was reported to affect matrix Gla protein (MGP), an inhibitor of bone morphogenetic proteins (BMPs), we studied BMP signaling and expression in various tissues of mice with and without functional ABCC. Enhanced BMP signaling was found in all examined tissues in the absence of ABCC6. Despite this, the expression of particular BMP proteins varied widely between tissues. Interestingly, the expression of most BMP proteins in the liver moved in the opposite direction to the same BMP proteins in kidneys in response to ABCC6 alterations. Thus, ABCC6 deficiency stimulates BMP signaling by acting on the expression of multiple BMPs. (C) 2015 The Authors. Published by Elsevier B.V.
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