Title :
Polypeptide GalNAc-transferase T3 and familial tumoral calcinosis
Abstract :
- Secretion of fibroblast growth factor 23 requires O-glycosylation
- Mutations in the gene encoding the glycosyltransferase polypeptide GalNAc-T3, which is involved in initiation of O-glycosylation, were recently identified as a cause of the rare autosomal recessive metabolic disorder familial tumoral calcinosis (OMIM 211900)
- Familial tumoral calcinosis is associated with hyperphosphatemia and massive ectopic calcifications
- Here, we demonstrate that the secretion of the phosphaturic factor fibroblast growth factor 23 ( FGF23 ) requires O-glycosylation, and that GalNAc-T3 selectively directs O-glycosylation in a subtilisin-like proprotein convertase recognition sequence motif , which blocks processing of FGF23
- The study suggests a novel posttranslational regulatory model of FGF23 involving competing O-glycosylation and protease processing to produce intact FGF23
Output (sent_index, trigger,
protein,
sugar,
site):
- 4. O-glycosylation, , -, -, motif
Output(Part-Of) (sent_index,
protein,
site):
*Output_Site_Fusion* (sent_index,
protein,
sugar,
site):