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While hypomineralization of dentin and odontoblast (OD) differentiation defects are known to occur in HPP, the underlying pathophysiology remains poorly understood.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Materials and methods<\/jats:title>\n                    <jats:p>We generated induced pluripotent stem cell (iPSC) lines from patients with perinatal severe HPP (Perinatal) and then isogenic (Rescued) and odontohypophosphatasia type (Odonto) lines using gene editing. These three HPP-iPSC lines were differentiated into OD-like cells via mesenchymal stem cells derived from neural crest cells. The characteristics of the OD-like cells were assessed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Rescued-OD-like cells demonstrated mineralization ability, increased\n                      <jats:italic>microtubule-associated protein tau<\/jats:italic>\n                      (\n                      <jats:italic>MAPT<\/jats:italic>\n                      ) expression, and unidirectional cell processes, while these characteristics were impaired in Perinatal- and Odonto-OD-like cells. These findings suggest that these features are associated with reduced ALP activity. Notably,\n                      <jats:italic>neurofilament light chain<\/jats:italic>\n                      (\n                      <jats:italic>NEFL<\/jats:italic>\n                      ) expression was enhanced in Odonto-OD-like cells compared to Perinatal- and Rescued-OD-like cells.\n                      <jats:italic>NEFL<\/jats:italic>\n                      knockdown partially rescued cell process elongation in Odonto-OD-like cells without affecting alkaline phosphatase activity, while\n                      <jats:italic>NEFL<\/jats:italic>\n                      overexpression inhibited cell process elongation in Rescued-OD-like cells.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>\n                      Enhanced expression of\n                      <jats:italic>NEFL<\/jats:italic>\n                      in Odonto-OD-like cells negatively correlates with OD morphology and\u00a0may be relevant to odontoblast morphological abnormalities associated with odontohypophosphatasia; however, generalization to other odonto-HPP genotypes requires additional patient-derived lines.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s00774-026-01703-5","type":"journal-article","created":{"date-parts":[[2026,3,14]],"date-time":"2026-03-14T08:19:19Z","timestamp":1773476359000},"page":"429-441","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["NEFL is associated with inhibition of odontoblastic process in odontohypophosphatasia"],"prefix":"10.1007","volume":"44","author":[{"given":"Akira","family":"Nozoe","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasuhisa","family":"Ohata","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Makoto","family":"Fujiwara","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenichi","family":"Yamamoto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toshihiko","family":"Nambara","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiho","family":"Nakano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazuaki","family":"Miyagawa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mikihiko","family":"Kogo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takeshi","family":"Taketani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takuo","family":"Kubota","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasuji","family":"Kitabatake","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susumu","family":"Tanaka","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6517-8825","authenticated-orcid":false,"given":"Keiichi","family":"Ozono","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,3,14]]},"reference":[{"key":"1703_CR1","doi-asserted-by":"publisher","first-page":"439","DOI":"10.1210\/edrv-15-4-439","volume":"15","author":"MP Whyte","year":"1994","unstructured":"Whyte MP (1994) Hypophosphatasia and the role of alkaline phosphatase in skeletal mineralization (in eng). 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K O. received payments from Kyowa Kirin, Alexion Pharma, and Novo Nordisk Pharma. The other authors declare no conflicts of interest.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"The present study, including the experimental procedures for skin biopsy and iPSC production, was approved by the Osaka University Graduate School of Medicine and Shimane University Medical Hospital ethical committee [approval numbers: 13123 (823)-4 and 1110].","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}},{"value":"Written informed consent was obtained from the parents or legal guardians of the patients in accordance with the Declaration of Helsinki.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Informed consent"}}]}}