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ey0019.5-11 | Translational highlights | ESPEYB19

5.11. A reference range for plasma levels of inorganic pyrophosphate in children using the ATP sulfurylase method

E Bernhard , Y Nitschke , G Khursigara , Y Sabbagh , Y Wang , F Rutsch

J Clin Endocrinol Metab. 2022 Jan 1;107(1):109-118.Abstract: https://pubmed-ncbi-nlm-nih-gov/34498693/In Brief: This study established a standard range of Inorganic Pyrophosphate (PPi) between 2.36 and 4.44 µM (5th-95th percentiles) in the blood plasma of children and adolescents aged 0 to 18 years, using the ATP sulfurylase assay. There was no sex difference and the range is similar t...

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ey0019.5-10 | Translational highlights | ESPEYB19

5.10. INZ-701 prevents ectopic tissue calcification and restores bone architecture and growth in ENPP1-deficient mice

Z Cheng , K O'Brien , J Howe , C Sullivan , D Schrier , A Lynch , S Jungles , Y Sabbagh , D Thompson

J Bone Miner Res. 2021 Aug;36(8):1594-1604.Abstract: https://pubmed-ncbi-nlm-nih-gov/33900645/In Brief: Enzyme replacement therapy with human ENPP1-Fc protein in Enpp1asj/asj mice, a murine model of ENPP1 deficiency, restored circulating levels of PPi, prevented clinical manifestations and decreased mortality.Commentary: ENPP1 deficiency causes gen...

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ESPE Yearbook of Paediatric Endocrinology

5.11. A reference range for plasma levels of inorganic pyrophosphate in children using the ATP sulfurylase method

5.10. INZ-701 prevents ectopic tissue calcification and restores bone architecture and growth in ENPP1-deficient mice

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