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Dental pulp stem cells as a promising model to study imprinting diseases

International Journal of Oral science 2022年第2期14卷 174-181页

作者： Eloïse Giabicani Aurélie Pham Céline sélénou Marie-Laure sobrier Caroline Andrique Julie Lesieur agnès linglart Anne Poliard Catherine Chaussain Irène Netchine Universitéde Paris URP2496Laboratoire Pathologiesimagerie et biothérapies oro-facialesMontrougeFrance INsERM UMRS_938 Centre de Recherche Saint AntoineParisFrance APHP Hôpital de BicêtreEndocrinologie et Diabétologie de l’EnfantCentre de Référence des Maladies Rares du Métabolisme du Calcium et du PhosphateLe Kremlin-BicêtreFrance APHP Hôpital BretonneauCentre de Référence des Maladies Rares du Métabolisme du Calcium et du Phosphate et FHU DDS-netParisFrance sorbonne Université INSERMUMRS_938APHPHôpital Armand TrousseauExplorations Fonctionnelles EndocriniennesParisFrance

Parental imprinting is an epigenetic process leading to monoallelic expression of certain genes depending on their parental *** diseases are characterized by growth and metabolic issues starting from birth to *** are mainly due to methylation defects in imprinting control region that drive the abnormal expression of imprinted *** currently lack relevant animal or cellular models to unravel the pathophysiology of growth failure in these *** aimed to characterize the methylation of imprinting regions in dental pulp stem cells and during their differentiation in osteogenic cells(involved in growth regulation)to assess the interest of this cells in modeling imprinting *** collected dental pulp stem cells from five controls and four patients(three with silver-Russell syndrome and one with Beckwith-Wiedemann syndrome).Methylation analysis of imprinting control regions involved in these syndromes showed a normal profile in controls and the imprinting defect in *** results were maintained in dental pulp stem cells cultured under osteogenic ***,we confirmed the same pattern in six other loci involved in imprinting diseases in *** also confirmed monoallelic expression of H19(an imprinted gene)in controls and its biallelic expression in one *** imprinting control regions methylation analysis shows the strong potential of dental pulp stem cells in modeling imprinting diseases,in which imprinting regions are preserved in culture and during osteogenic *** will allow to perform in vitro functional and therapeutic tests in cells derived from dental pulp stem cells and generate other cell-types.

关键词： pulp maintained fir

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维普期刊数据库

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Dental impact of anti-fibroblast growth factor 23 therapy in X-linked hypophosphatemia

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International Journal of Oral science 2023年第4期15卷 691-700页

作者： Elis J.Lira dos santos Kenta Nakajima Julien Po Ayako Hanai Volha Zhukouskaya Martin Biosse Duplan agnès linglart Takashi shimada Catherine Chaussain Claire Bardet UniversitéParis Cité Institut des maladies musculo-squelettiquesLaboratory Orofacial PathologiesImaging and Biotherapies URP2496 and FHU-DDS-NetDental Schooland Plateforme d’Imagerie du Vivant(PIV)MontrougeFrance R&D Division Kyowa KirinCo.Ltd3-6-6 Asahi-machiMachida-shiTokyoJapan UniversitéParis Cité Institut ImagineINSERM UMR 1163ParisFrance AP-HP Reference Center for Rare Disorders of the Calcium and Phosphate MetabolismDental Medicine DepartmentBretonneau HospitalGHN-UniversitéParis CitéParisFrance Paris-saclay University AP-HPINSERM U1185DMU SEAEndocrinology and Diabetes for ChildrenReference Center for Rare Diseases of the Calcium and Phosphate MetabolismOSCAR filièreEndoRareand BOND ERNsBicêtre Paris Saclay HospitalLe Kremlin-BicêtreFrance Medical Affairs Department Kyowa KirinCo.Ltd1-9-2 OtemachiChiyoda-kuTokyoJapan

Elevated fibroblast growth factor 23(FGF23)in X-linked hypophosphatemia(XLH)results in rickets and phosphate wasting,manifesting by severe bone and dental ***,a FGF23-neutralizing antibody,an alternative to conventional treatment(phosphorus and active vitamin D analogs),showed significant improvement in the long bone ***,we examined whether FGF23 antibody(FGF23-mAb)also improved the dentoalveolar features associated with ***-week-old male Hyp mice were injected weekly with 4 or 16 mg·kg−1 of FGF23-mAb for 2 months and compared to wild-type(WT)and vehicle(PBs)treated Hyp mice(n=3–7 mice).Micro-CT analyses showed that both doses of FGF23-mAb restored dentin/cementum volume and corrected the enlarged pulp volume in Hyp mice,the higher concentration resulting in a rescue similar to WT ***23-mAb treatment also improved alveolar bone volume fraction and mineral density compared to vehicle-treated *** revealed improved mineralization of the dentoalveolar tissues,with a decreased amount of osteoid,predentin and *** periodontal ligament attachment was also observed,evidenced by restoration of the acellular *** preclinical data were consistent with the retrospective analysis of two patients with XLH showing that burosumab treatment improved oral *** together,our data show that the dentoalveolar tissues are greatly improved by FGF23-mAb treatment,heralding its benefit in clinics for dental abnormalities.

关键词： doses vitamin treatment

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一个可能为发作性运动障碍家族的Ib型假性甲状旁腺功能减退症的分子学诊断

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世界核心医学期刊文摘（儿科学分册） 2006年第8期2卷 58-58页

作者： Mahmud F.H. linglart A. Bastepe M. A.N.Lteif 俞晓梅 Mayo Clinic 200 First St SWRochesterMN55905United States Dr.

We describe 2 sisters diagnosed initially with paroxysmal kinesigenic choreoat hetosis,a condition characterized by brief episodes of spasms precipitated by s udden ***,subsequent testing showed hypocalcemia,hyperphosphatemi a,and elevated parathyroid hormone levels consistent with pseudohypoparathyroid ism type *** diagnosis was confirmed by genetic testing,which identified a 3-kilobase deletion on chromosome *** report describes the neurologic presentation,metabolic derangement,and underlying genetic mutation in a famil *** also reinforces the importance of metabolic testing in the evaluation of p ediatric patients with movement disorders.

关键词：假性甲状旁腺功能减退症发作性运动障碍分子学诊断家族成员 b型舞蹈手足徐动症高磷酸盐血症神经系统表现基因测试代谢紊乱

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