{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T23:02:00Z","timestamp":1790550120484,"version":"4.1.0"},"reference-count":48,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2020,6,10]],"date-time":"2020-06-10T00:00:00Z","timestamp":1591747200000},"content-version":"vor","delay-in-days":9,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["faseb.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["FASEB BioAdvances"],"published-print":{"date-parts":[[2020,6]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Low bone mineral density (BMD)\u2014diagnosed as osteoporosis or osteopenia\u2014has been reported as a new characteristic feature of Fabry disease; however, the mechanism underlying the development of low BMD is unknown. We previously revealed that a mouse model of Fabry disease [\n                    <jats:italic>\n                      Gla\n                      <jats:sup>tm<\/jats:sup>\n                      Tg(CAG\u2010A4GALT)\n                    <\/jats:italic>\n                    ] exhibits impaired functioning of medullary thick ascending limb (mTAL), leading to insufficient Ca\n                    <jats:sup>2+<\/jats:sup>\n                    reabsorption and hypercalciuria. Here, we investigated bone metabolism in\n                    <jats:italic>\n                      Gla\n                      <jats:sup>tm<\/jats:sup>\n                      Tg(CAG\u2010A4GALT)\n                    <\/jats:italic>\n                    mice without marked glomerular or proximal tubular damage. Low BMD was detected by 20\u00a0weeks of age\n                    <jats:italic>via<\/jats:italic>\n                    micro\u2010X\u2010ray\u2010computed tomography. Bone histomorphometry revealed that low BMD results by accelerated bone resorption and osteomalacia. Plasma parathyroid hormone levels increased in response to low blood Ca\n                    <jats:sup>2+<\/jats:sup>\n                    \u2014not plasma fibroblast growth factor 23 (FGF\u201023) elevation\u2014by 5\u00a0weeks of age and showed progressively increased phosphaturic action. Secondary hyperparathyroidism developed by 20\u00a0weeks of age and caused hyperphosphatemia, which increased plasma FGF\u201023 levels with phosphaturic action. The expression of 1\u03b1\u2010hydroxylase [synthesis of 1\u03b1,25(OH)\n                    <jats:sub>2<\/jats:sub>\n                    D\n                    <jats:sub>3<\/jats:sub>\n                    ] in the kidney did not decrease, but that of 24\u2010hydroxylase [degradation of 1\u03b1,25(OH)\n                    <jats:sub>2<\/jats:sub>\n                    D\n                    <jats:sub>3<\/jats:sub>\n                    ] decreased. Vitamin D deficiency was ruled out as the cause of osteomalacia, as plasma 1\u03b1,25(OH)\n                    <jats:sub>2<\/jats:sub>\n                    D\n                    <jats:sub>3<\/jats:sub>\n                    and 25(OH)D\n                    <jats:sub>3<\/jats:sub>\n                    levels were maintained. Results demonstrate that secondary hyperparathyroidism due to mTAL impairment causes accelerated bone resorption and osteomalacia due to hyperphosphaturia and hypercalciuria, leading to low BMD in Fabry model mice.\n                  <\/jats:p>","DOI":"10.1096\/fba.2019-00080","type":"journal-article","created":{"date-parts":[[2020,4,30]],"date-time":"2020-04-30T17:23:35Z","timestamp":1588267415000},"page":"365-381","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Low bone mineral density due to secondary hyperparathyroidism in the\n                    <i>\n                      Gla\n                      <sup>tm<\/sup>\n                      Tg(CAG\u2010A4GALT)\n                    <\/i>\n                    mouse model of Fabry disease"],"prefix":"10.1096","volume":"2","author":[{"given":"Hiroki","family":"Maruyama","sequence":"first","affiliation":[{"name":"Department of Clinical Nephroscience Niigata University Graduate School of Medical and Dental Sciences Niigata Niigata Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Atsumi","family":"Taguchi","sequence":"additional","affiliation":[{"name":"Department of Clinical Nephroscience Niigata University Graduate School of Medical and Dental Sciences Niigata Niigata Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariko","family":"Mikame","sequence":"additional","affiliation":[{"name":"Department of Clinical Nephroscience Niigata University Graduate School of Medical and Dental Sciences Niigata Niigata Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hongmei","family":"Lu","sequence":"additional","affiliation":[{"name":"Laboratory of Genome Research Research Institute for Diseases of Old Age Juntendo University Graduate School of Medicine Bunkyo\u2010ku Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Norihiro","family":"Tada","sequence":"additional","affiliation":[{"name":"Laboratory of Genome Research Research Institute for Diseases of Old Age Juntendo University Graduate School of Medicine Bunkyo\u2010ku Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Muneaki","family":"Ishijima","sequence":"additional","affiliation":[{"name":"Department of Medicine for Orthopaedics and Motor Organ Juntendo University Graduate School of Medicine Bunkyo\u2010ku Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Haruka","family":"Kaneko","sequence":"additional","affiliation":[{"name":"Department of Medicine for Orthopaedics and Motor Organ Juntendo University Graduate School of Medicine Bunkyo\u2010ku Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariko","family":"Kawai","sequence":"additional","affiliation":[{"name":"Department of Pharmacology Osaka Dental University Hirakata Osaka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sawako","family":"Goto","sequence":"additional","affiliation":[{"name":"Department of Applied Molecular Medicine Niigata University Graduate School of Medical and Dental Sciences Niigata Niigata Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akihiko","family":"Saito","sequence":"additional","affiliation":[{"name":"Department of Applied Molecular Medicine Niigata University Graduate School of Medical and Dental Sciences Niigata Niigata Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Riuko","family":"Ohashi","sequence":"additional","affiliation":[{"name":"Histopathology Core Facility Faculty of Medicine Niigata University Niigata Niigata Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuji","family":"Nishikawa","sequence":"additional","affiliation":[{"name":"Division of Tumor Pathology Department of Pathology Asahikawa Medical University Asahikawa Hokkaido Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoshi","family":"Ishii","sequence":"additional","affiliation":[{"name":"Department of Matrix Medicine Faculty of Medicine Oita University Yufu Oita Japan"},{"name":"Biochemical Laboratory GlycoPharma Corporation Oita Oita Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2020,6,10]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM196705252762101"},{"key":"e_1_2_8_3_1","unstructured":"The Human Gene Mutation Database.http:\/\/www.hgmd.cf.ac.uk\/ac\/gene.php?gene=GLA. 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