ISSN 1662-4009 (online)

ey0015.4-13 | New paradigms | ESPEYB15

4.13 An essential role for IGF2 in cartilage development and glucose metabolism during postnatal long bone growth

T Uchimura , JM Hollander , DS Nakamura , Z Liu , CJ Rosen , I Georgakoudi , L Zeng

To read the full abstract: Development 2017; 144:3533-3546Endochondral ossification is the process by which the embryonic cartilaginous scaffold of most bones is gradually replaced by bone. During this process chondrocytes proliferate, undergo hypertrophy and maturation and form the growth plate, essential for bone growth. Postnatal bone growth is tightly regulated by both systemic and lo...

ey0018.5-2 | Novel treatments for rare skeletal disorders | ESPEYB18

5.2. Evaluation of FGFR inhibitor ASP5878 as a drug candidate for achondroplasia

Ozaki Tomonori , Kawamoto Tatsuya , Iimori Yuki , Takeshita Nobuaki , Yamagishi Yukiko , Nakamura Hiroaki , Kamohara Masazumi , Fujita Kaori , Tanahashi Masayuki , Tsumaki Noriyuki

Sci Rep. 2020 Dec 1;10(1):20915 Abstract: https://pubmed.ncbi.nlm.nih.gov/33262386/In brief: Inhibition of excessive FGFR3 signalling constitutes the key mechanism of pharmacological treatments in achondroplasia. In this pharmacokinetic and pharmacodynamic animal study, the FGFR inhibitor ASP5878 with potential oral application mode revealed beneficial effects on skeletal growth in...

ey0020.2-14 | New Perspectives | ESPEYB20

2.14. Pathogenic copy number and sequence variants in children born SGA with short stature without imprinting disorders

K Hara-Isono , A Nakamura , T Fuke , T Inoue , S Kawashima , K Matsubara , S Sano , K Yamazawa , M Fukami , T Ogata , M Kagami

Brief summary: This observational study aimed at clarifying the contribution of pathogenic copy number variants (PCNVs) and candidate pathogenic variants in 86 children born small-for-gestational-age with short stature (SGA-SS).Approximately 10% of children born SGA do not show catch-up and remain permanently short (SGA-SS) (1,2). There is increasing evidence suggesting that genetic abnormalities underlie a high proportion of SGA-SS children. In this stu...

ey0020.3-18 | Advances in Growth, Bone Biology, and Mineral Metabolism | ESPEYB20

3.18. Periosteal stem cells control growth plate stem cells during postnatal skeletal growth

M Tsukasaki , N Komatsu , T Negishi-Koga , NC Huynh , R Muro , Y Ando , Y Seki , A Terashima , W Pluemsakunthai , T Nitta , T Nakamura , T Nakashima , S Ohba , H Akiyama , K Okamoto , R Baron , H Takayanagi

In Brief: The authors use cell tracing approaches in a PRMT5flox/Δ Ctsk-Cre strain to label periosteal stem cells (PSCs) and show that PSCs are not only essential for intramembranous bone formation but also for endochondral bone formation. The study identifies the role of PSC derived indian hedgehog (Ihh) in maintaining growth plate homeostasis and postnatal skeletal growth.Commentary: The skeleton harbors stem cells in three independent ...