Impaired osteogenesis in Menkes disease-derived induced pluripotent stem cells
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. We present in vitro modeling for impaired osteogenesis in MD using...
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| Published in: | Stem cell research & therapy Vol. 6; no. 1; p. 160 |
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| Abstract | 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.
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.
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.
Our findings indicate that ATP7A dysfunction contributes to retardation in MSC development and impairs osteogenesis in MD. |
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| AbstractList | 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. 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. 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. 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. Our findings indicate that ATP7A dysfunction contributes to retardation in MSC development and impairs osteogenesis in MD. 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. 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. 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. Our findings indicate that ATP7A dysfunction contributes to retardation in MSC development and impairs osteogenesis in MD. INTRODUCTIONBone 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.METHODSWe 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.RESULTSThe 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.CONCLUSIONSOur findings indicate that ATP7A dysfunction contributes to retardation in MSC development and impairs osteogenesis in MD. |
| ArticleNumber | 160 |
| Audience | Academic |
| Author | Choi, Jin-Ho Choi, Jieun Kim, Dongkyu Lee, Beom Hee Han, Kyu-Min Han, Yong-Mahn Yoo, Han-Wook |
| Author_xml | – sequence: 1 givenname: Dongkyu surname: Kim fullname: Kim, Dongkyu email: kdkd86@kaist.ac.kr organization: Department of Biological Science, Korea Advanced Institute of Science Technology (KAIST), Daejeon, 305-701, Republic of Korea. kdkd86@kaist.ac.kr – sequence: 2 givenname: Jieun surname: Choi fullname: Choi, Jieun email: euny519@kaist.ac.kr organization: Department of Biological Science, Korea Advanced Institute of Science Technology (KAIST), Daejeon, 305-701, Republic of Korea. euny519@kaist.ac.kr – sequence: 3 givenname: Kyu-Min surname: Han fullname: Han, Kyu-Min email: hankm88@kaist.ac.kr organization: Department of Biological Science, Korea Advanced Institute of Science Technology (KAIST), Daejeon, 305-701, Republic of Korea. hankm88@kaist.ac.kr – sequence: 4 givenname: Beom Hee surname: Lee fullname: Lee, Beom Hee email: bhlee@amc.seoul.kr organization: Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, South Korea. bhlee@amc.seoul.kr – sequence: 5 givenname: Jin-Ho surname: Choi fullname: Choi, Jin-Ho email: jhc@amc.seoul.kr organization: Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, South Korea. jhc@amc.seoul.kr – sequence: 6 givenname: Han-Wook surname: Yoo fullname: Yoo, Han-Wook email: hwyoogene@gmail.com organization: Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, South Korea. hwyoogene@gmail.com – sequence: 7 givenname: Yong-Mahn surname: Han fullname: Han, Yong-Mahn email: ymhan57@kaist.ac.kr organization: Department of Biological Science, Korea Advanced Institute of Science Technology (KAIST), Daejeon, 305-701, Republic of Korea. ymhan57@kaist.ac.kr |
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| Snippet | Bone abnormalities, one of the primary manifestations of Menkes disease (MD), include a weakened bone matrix and low mineral density. However, the molecular... Introduction Bone abnormalities, one of the primary manifestations of Menkes disease (MD), include a weakened bone matrix and low mineral density. However, the... INTRODUCTIONBone abnormalities, one of the primary manifestations of Menkes disease (MD), include a weakened bone matrix and low mineral density. However, the... |
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| SubjectTerms | Adenosine Triphosphatases - genetics Animals Antigens, CD - genetics Antigens, CD - metabolism Bones Cation Transport Proteins - genetics Cells, Cultured Comparative analysis Copper-transporting ATPases Density Endoglin Extracellular Matrix - metabolism Genetic aspects Humans Induced Pluripotent Stem Cells - cytology Induced Pluripotent Stem Cells - metabolism Induced Pluripotent Stem Cells - transplantation Infant Infant, Newborn Menkes Kinky Hair Syndrome - genetics Menkes Kinky Hair Syndrome - metabolism Menkes Kinky Hair Syndrome - pathology Mice Mutation Osteogenesis Phosphatases Physiological aspects Protein-Lysine 6-Oxidase - metabolism Receptors, Cell Surface - genetics Receptors, Cell Surface - metabolism Stem cells |
| Title | Impaired osteogenesis in Menkes disease-derived induced pluripotent stem cells |
| URI | https://www.ncbi.nlm.nih.gov/pubmed/26347346 https://www.proquest.com/docview/1772853472 https://search.proquest.com/docview/1710654958 https://pubmed.ncbi.nlm.nih.gov/PMC4562349 |
| Volume | 6 |
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