Mannose 6-phosphate-dependent targeting of lysosomal enzymes is required for normal craniofacial and dental development
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- 作者：Till Koehnea ; b ; 1 ; Sandra Markmannc ; 1 ; Michaela Schweizerd ; Nicole Muscholc ; Reinhard E. Friedriche ; Christian Hagelf ; Markus Glatzelf ; Bä ; rbel Kahl-Niekea ; Michael Amlingb ; Thorsten Schinkeb ; Thomas Braulkec ; braulke@uke.de" class="auth_mail" title="E-mail the corresponding author
- 关键词：Cementoblasts ; Craniofacial anomalies ; Gingival hypertrophy ; Lysosomes ; Mucolipidosis II ; Osteoclastogenesis
- 刊名：Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease
- 出版年：2016
- 出版时间：September 2016
- 年：2016
- 卷：1862
- 期：9
- 页码：1570-1580
- 全文大小：2580 K
文摘
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MLII mice closely mimic the craniofacial and dental phenotype of MLII patients.
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Increased osteoclastogenesis causes alveolar bone loss in MLII.
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Missorting of lysosomal enzymes leads to hypercementosis.
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Storage material and disturbed collagen synthesis result in gingival hypertrophy.
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Mannose 6-phosphate-dependent lysosomal targeting controls periodontal homeostasis.