Searchable abstracts of presentations at key conferences on calcified tissues

ba0001pp460 | Other diseases of bone and mineral metabolism | ECTS2013

Osteopontin ASARM peptide binding to crystal faces of hydroxyapatite – computational simulations

Mansouri Ahmad , Masica David , Gray Jeffrey , McKee Marc

ASARM peptide (acidic, serine- and aspartate-rich motif) and osteopontin (OPN) fragments accumulate in X-linked hypophosphatemia patients and/or in the Hyp mouse model and, when phosphorylated, potently inhibit mineralization in osteoblast cultures. To investigate this inhibition, we modeled the binding to hydroxyapatite of the human OPN-ASARM peptide (DDSHQSDESHHSDESDEL) using RosettaSurface computational simulations. Peptide binding to hydroxyapatite atomic planes constructe...

ba0003pp111 | Cell biology: osteoblasts and bone formation | ECTS2014

Osteopontin as a novel substrate for proprotein convertase 5/6 (PCSK5) in bone

Hoac Betty , Susan-Resiga Delia , Essalmani Rachid , Marcinkiweicz Edwidge , Barros Nilana , Seidah Nabil , McKee Marc

Proprotein convertase PC5/6 (Pcsk5) is expressed in mouse bone and Pcsk5 epiblast-specific conditional knockout mice have a bone phenotype displaying small size, delayed ossification and additional thoracic segments and ribs. Some features are attributed to growth and developmental factor 11 (GDF11) – a known substrate for PC5/6 – while the delayed mineralization has yet to be explained. Osteopontin (OPN) is a bone matrix protein with roles in miner...

ba0001pp469 | Other diseases of bone and mineral metabolism | ECTS2013

MEPE-derived ASARM peptide impairs mineralization in tooth models of X-linked hypophosphatemia

Salmon Benjamin , Bardet Claire , Khaddam Mayssam , Baroukh Brigitte , Lesieur Julie , Denmat Dominique Le , Nicoletti Antonino , Poliard Anne , Rowe Peter S , Linglart Agnes , McKee Marc D , Chaussain Catherine

Mutations in the PHEX gene cause X-linked familial hypophosphatemic rickets (XLH) with severe bone (osteomalacia) and tooth abnormalities being the distinguishing features of this disease. The PHEX mutations lead to an increase in ASARM peptides (acidic serine- and aspartate-rich motif) and osteopontin fragments which inhibit bone extracellular matrix mineralization. MEPE-derived ASARM has been shown to accumulate in tooth dentin of patients with XLH where it may impair dentin...