論文 - 詳細
| RRC ID | 81117 |
|---|---|
| 著者 | Nakamura Y, Kobayashi H, Fukuda N, Tanaka S, Murata Y, Hatanaka Y, Haketa A, Tsunemi A, Chen L, Abe M. |
| タイトル | Induced pluripotent stem cells derived renal tubular cells from a patient with pseudohypoparathyroidism and its response to parathyroid hormone stimulation. |
| ジャーナル | Mol Biol Rep |
| Abstract |
INTRODUCTION:Creating induced pluripotent stem cells (iPSCs) from somatic cells of patients with genetic diseases offers a pathway to generate disease-specific iPSCs carrying genetic markers. Differentiating these iPSCs into renal tubular cells can aid in understanding the pathophysiology of rare inherited renal tubular diseases through cellular experiments. MATERIALS AND METHODS:Two Japanese patients with Pseudohypoparathyroidism (PHP), a 49-year-old woman and a 71-year-old man, were studied. iPSC-derived tubular cells were established from their peripheral blood mononuclear cells (PBMCs). We examined changes in intracellular and extracellular cyclic adenosine monophosphate (cAMP) levels in these cells in response to parathyroid hormone (PTH) stimulation. RESULTS:Renal tubular cells, differentiated from iPSCs of a healthy control (648A1), showed a PTH-dependent increase in both intracellular and extracellular cAMP levels. However, the renal tubular cells derived from the PHP patients' iPSCs showed inconsistent changes in cAMP levels upon PTH exposure. CONCLUSION:We successfully created disease-specific iPSCs from PHP patients' PBMCs, differentiated them into tubular cells, and replicated the distinctive response of the disease to PTH in vitro. This approach could enhance our understanding of the pathophysiology of inherited renal tubular diseases and contribute to developing effective treatments. |
| 巻・号 | 51(1) |
| ページ | 790 |
| 公開日 | 2024-7-11 |
| DOI | 10.1007/s11033-024-09751-8 |
| PII | 10.1007/s11033-024-09751-8 |
| PMID | 38990390 |
| MeSH | Aged Cell Differentiation* / drug effects Cells, Cultured Cyclic AMP* / metabolism Female Humans Induced Pluripotent Stem Cells* / metabolism Kidney Tubules* / metabolism Kidney Tubules* / pathology Leukocytes, Mononuclear* / metabolism Male Middle Aged Parathyroid Hormone* / metabolism Parathyroid Hormone* / pharmacology Pseudohypoparathyroidism* / genetics Pseudohypoparathyroidism* / metabolism |
| IF | 1.402 |
| リソース情報 | |
| ヒト・動物細胞 | 648A1(HPS0360) |