DC Field | Value | Language |
---|---|---|
dc.contributor.author | Kim, Dongkyu | ko |
dc.contributor.author | Choi, Jieun | ko |
dc.contributor.author | Han, Kyu-Min | ko |
dc.contributor.author | Lee, Beom Hee | ko |
dc.contributor.author | Choi, Jin-Ho | ko |
dc.contributor.author | Yoo, Han-Wook | ko |
dc.contributor.author | Han, Yong-Mahn | ko |
dc.date.accessioned | 2016-04-15T03:00:30Z | - |
dc.date.available | 2016-04-15T03:00:30Z | - |
dc.date.created | 2015-10-02 | - |
dc.date.created | 2015-10-02 | - |
dc.date.issued | 2015-09 | - |
dc.identifier.citation | STEM CELL RESEARCH & THERAPY, v.6 | - |
dc.identifier.issn | 1757-6512 | - |
dc.identifier.uri | http://hdl.handle.net/10203/203899 | - |
dc.description.abstract | Introduction: Bone abnormalities, one of the primary manifestations of Menkes disease (MD), include a weakened bone matrix and low mineral density. However, the molecular and cellular mechanisms underlying these bone defects are poorly understood. Methods: We present in vitro modeling for impaired osteogenesis in MD using human induced pluripotent stem cells (iPSCs) with a mutated ATP7A gene. MD-iPSC lines were generated from two patients harboring different mutations. Results: The MD-iPSCs showed a remarkable retardation in CD105 expression with morphological anomalies during development to mesenchymal stem cells (MSCs) compared with wild-type (WT)-iPSCs. Interestingly, although prolonged culture enhanced CD105 expression, mature MD-MSCs presented with low alkaline phosphatase activity, reduced calcium deposition in the extracellular matrix, and downregulated osteoblast-specific genes during osteoblast differentiation in vitro. Knockdown of ATP7A also impaired osteogenesis in WT-MSCs. Lysyl oxidase activity was also decreased in MD-MSCs during osteoblast differentiation. Conclusions: Our findings indicate that ATP7A dysfunction contributes to retardation in MSC development and impairs osteogenesis in MD. | - |
dc.language | English | - |
dc.publisher | BIOMED CENTRAL LTD | - |
dc.subject | LYSYL OXIDASE ACTIVITY | - |
dc.subject | KINKY-HAIR SYNDROME | - |
dc.subject | COPPER | - |
dc.subject | DIFFERENTIATION | - |
dc.subject | BONE | - |
dc.subject | EXPRESSION | - |
dc.subject | HYPOPHOSPHATASIA | - |
dc.subject | MINERALIZATION | - |
dc.subject | TRAFFICKING | - |
dc.subject | OSTEOBLASTS | - |
dc.title | Impaired osteogenesis in Menkes disease-derived induced pluripotent stem cells | - |
dc.type | Article | - |
dc.identifier.wosid | 000360787400001 | - |
dc.identifier.scopusid | 2-s2.0-84940823595 | - |
dc.type.rims | ART | - |
dc.citation.volume | 6 | - |
dc.citation.publicationname | STEM CELL RESEARCH & THERAPY | - |
dc.identifier.doi | 10.1186/s13287-015-0147-5 | - |
dc.contributor.localauthor | Han, Yong-Mahn | - |
dc.contributor.nonIdAuthor | Lee, Beom Hee | - |
dc.contributor.nonIdAuthor | Choi, Jin-Ho | - |
dc.contributor.nonIdAuthor | Yoo, Han-Wook | - |
dc.description.isOpenAccess | Y | - |
dc.type.journalArticle | Article | - |
dc.subject.keywordPlus | LYSYL OXIDASE ACTIVITY | - |
dc.subject.keywordPlus | KINKY-HAIR SYNDROME | - |
dc.subject.keywordPlus | COPPER | - |
dc.subject.keywordPlus | DIFFERENTIATION | - |
dc.subject.keywordPlus | BONE | - |
dc.subject.keywordPlus | EXPRESSION | - |
dc.subject.keywordPlus | HYPOPHOSPHATASIA | - |
dc.subject.keywordPlus | MINERALIZATION | - |
dc.subject.keywordPlus | TRAFFICKING | - |
dc.subject.keywordPlus | OSTEOBLASTS | - |
Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.