tailieunhanh - Báo cáo y học: "P5L mutation in Ank results in an increase in extracellular inorganic pyrophosphate during proliferation and nonmineralizing hypertrophy in stably transduced ATDC5 cells"

Tuyển tập các báo cáo nghiên cứu về y học được đăng trên tạp chí y học General Psychiatry cung cấp cho các bạn kiến thức về ngành y đề tài: P5L mutation in Ank results in an increase in extracellular inorganic pyrophosphate during proliferation and nonmineralizing hypertrophy in stably transduced ATDC5 cells. | Available online http content 8 6 R164 Open Access Research article P5L mutation in Ank results in an increase in extracellular inorganic pyrophosphate during proliferation and nonmineralizing hypertrophy in stably transduced ATDC5 cells Raihana Zaka1 David Stokes1 Arnold S Dion2 Anna Kusnierz1 Fei Han1 and Charlene J Williams1 1 Division of Rheumatology Department of Medicine Thomas Jefferson University Philadelphia PA 19107 USA 2College of Graduate Studies Thomas Jefferson University Philadelphia PA 191 07 USA Corresponding author Charlene J Williams Received 10 Aug 2006 Revisions requested 30 Aug 2006 Revisions received 5 Oct 2006 Accepted 26 Oct 2006 Published 26 Oct 2006 Arthritis Research Therapy 2006 8 R164 doi ar2072 This article is online at http content 8 6 R164 2006 Zaka et al. licensee BioMed Central Ltd. This is an open access article distributed under the terms of the Creative Commons Attribution License http licenses by which permits unrestricted use distribution and reproduction in any medium provided the original work is properly cited. Abstract Ank is a multipass transmembrane protein that regulates the cellular transport of inorganic pyrophosphate. In the progressive ankylosis ank mouse a premature termination mutation at glutamic acid 440 results in a phenotype characterized by inappropriate deposition of basic calcium phosphate crystals in skeletal tissues. Mutations in the amino terminus of ANKH the human homolog of Ank result in familial calcium pyrophosphate dihydrate deposition disease. It has been hypothesized that these mutations result in a gain-of-function with respect to the elaboration of extracellular inorganic pyrophosphate. To explore this issue in a mineralization-competent system we stably transduced ATDC5 cells with wild-type Ank as well as with familial chondrocalcinosis-causing Ank mutations. We evaluated the .

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