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| Content Provider | Springer Nature : BioMed Central |
|---|---|
| Author | Kim, Dongkyu Choi, Jieun Han, Kyu-Min Lee, Beom Hee Choi, Jin-Ho Yoo, Han-Wook Han, Yong-Mahn |
| 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. |
| Related Links | https://stemcellres.biomedcentral.com/counter/pdf/10.1186/s13287-015-0147-5.pdf |
| Ending Page | 12 |
| Page Count | 12 |
| Starting Page | 1 |
| File Format | HTM / HTML |
| ISSN | 17576512 |
| DOI | 10.1186/s13287-015-0147-5 |
| Journal | Stem Cell Research & Therapy |
| Issue Number | 1 |
| Volume Number | 6 |
| Language | English |
| Publisher | BioMed Central |
| Publisher Date | 2015-09-07 |
| Access Restriction | Open |
| Subject Keyword | Stem Cells Cell Biology Regenerative Medicine Tissue Engineering Biomedical Engineering and Bioengineering Alcian Blue Staining Menkes Disease ATP7A Gene Intracellular Copper ATP7A Mutation Regenerative Medicine/Tissue Engineering |
| Content Type | Text |
| Resource Type | Article |
| Subject | Cell Biology Medicine Biochemistry, Genetics and Molecular Biology Molecular Medicine |
| Journal Impact Factor | 7.1/2023 |
| 5-Year Journal Impact Factor | 7.9/2023 |
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