Platelet hyperreactivity explains the bleeding abnormality and macrothrombocytopenia in a murine model of sitosterolemia-Reference-Cited by-同舟云学术

Platelet hyperreactivity explains the bleeding abnormality and macrothrombocytopenia in a murine model of sitosterolemia

Published:2013-10-10 Issue:15 Volume:122 Page:2732-2742
ISSN:0006-4971
Container-title:Blood
language:en
Short-container-title:

Author:

Kanaji Taisuke¹,Kanaji Sachiko¹,Montgomery Robert R.¹²³,Patel Shailendra B.⁴⁵,Newman Peter J.¹⁶⁷⁸

Affiliation:

1. Blood Research Institute, BloodCenter of Wisconsin, Milwaukee, WI;

2. Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI;

3. Children's Research Institute, Children's Hospital of Wisconsin, Milwaukee, WI; and

4. Clement J. Zablocki Veterans Affairs Medical Center,

5. Division of Endocrinology, Metabolism, and Clinical Nutrition,

6. Pharmacology and

7. Cellular Biology, and

8. The Cardiovascular Research Center, Medical College of Wisconsin, Milwaukee, WI

Abstract

Key Points Plant sterol accumulation in platelet membrane induces platelet hyperreactivity. Internalization of the αIIbβ3 complex and filamin A degradation cause macrothrombocytopenia and bleeding phenotype.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Link

http://ashpublications.org/blood/article-pdf/122/15/2732/1369437/2732.pdf

Reference49 articles.

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3. Absorbability of beta-sitosterol in humans.;Gould;Metabolism,1969

4. β-sitosterolemia and xanthomatosis. A newly described lipid storage disease in two sisters.;Bhattacharyya;J Clin Invest,1974

5. Accumulation of dietary cholesterol in sitosterolemia caused by mutations in adjacent ABC transporters.;Berge;Science,2000

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