Catalysis‐Independent ENPP1 Protein Signaling Regulates Mammalian Bone Mass
| العنوان: | Catalysis‐Independent |
|---|---|
| المؤلفون: | Kristin Zimmerman, Xiaochen Liu, Simon von Kroge, Paul Stabach, Ethan R. Lester, Emily Y. Chu, Shivani Srivastava, Martha J. Somerman, Steven M. Tommasini, Björn Busse, Thorsten Schinke, Thomas O. Carpenter, Ralf Oheim, Demetrios T. Braddock |
| المصدر: | Journal of Bone and Mineral Research. 37:1733-1749 |
| بيانات النشر: | Wiley, 2022. |
| سنة النشر: | 2022 |
| مصطلحات موضوعية: | Mammals, Phosphoric Diester Hydrolases, Endocrinology, Diabetes and Metabolism, Bone and Bones, Catalysis, Phosphates, Fibroblast Growth Factors, Mice, Animals, Orthopedics and Sports Medicine, Familial Hypophosphatemic Rickets, Pyrophosphatases, Vascular Calcification, beta Catenin |
| الوصف: | Biallelic ectonucleotide pyrophosphatase/phosphodiesterase 1 (ENPP1) deficiency induces vascular/soft tissue calcifications in generalized arterial calcification of infancy (GACI), and low bone mass with phosphate-wasting rickets in GACI survivors (autosomal hypophosphatemic rickets type-2). ENPP1 haploinsufficiency induces early-onset osteoporosis and mild phosphate wasting in adults. Both conditions demonstrate the unusual combination of reduced accrual of skeletal mineral, yet excess and progressive heterotopic mineralization. ENPP1 is the only enzyme that generates extracellular pyrophosphate (PPi), a potent inhibitor of both bone and heterotopic mineralization. Life-threatening vascular calcification in ENPP1 deficiency is due to decreased plasma PPi; however, the mechanism by which osteopenia results is not apparent from an understanding of the enzyme's catalytic activity. To probe for catalysis-independent ENPP1 pathways regulating bone, we developed a murine model uncoupling ENPP1 protein signaling from ENPP1 catalysis, Enpp1 |
| تدمد: | 1523-4681 0884-0431 |
| DOI: | 10.1002/jbmr.4640 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::314e95b8fd71f70c02b69948a42477c8 https://doi.org/10.1002/jbmr.4640 |
| Rights: | CLOSED |
| رقم الانضمام: | edsair.doi.dedup.....314e95b8fd71f70c02b69948a42477c8 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 15234681 08840431 |
|---|---|
| DOI: | 10.1002/jbmr.4640 |