Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.1.3.1 (alkaline phosphatase)
 47,916 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We present the case of a 38-year-old man in whom extensive bilateral melorheostosis was associated with elevated serum alkaline phosphatase, swelling of the right foot and progressive deformity of the left hand, left leg and right foot. Radiography, computed tomography and bone scintigraphy were performed. Following treatment with bisphosphonate (30 mg/day of pamidronate for 6 days) infusion, the pain and swelling of his right foot showed improvement and his elevated serum alkaline phosphatase decreased.
 ...
PMID:Atypical form of active melorheostosis and its treatment with bisphosphonate. 1248 33

betaig-h3 is an extracellular matrix (ECM) protein induced by TGF-beta, and it has motifs interacting with the alpha3beta1, alphavbeta5, and alphavbeta3 integrins. Our previous study shows the role of betaig-h3 in osteoblast differentiation and its involvement in melorheostosis, a rare bone disease. Here we demonstrate that betaig-h3 expression is down-regulated during the early stage of differentiation of the murine preosteoblastic cell line, KS483. The recombinant betaig-h3 and its FAS1 domain significantly inhibited in vitro osteoblast differentiation as evaluated by matrix mineralization/bone nodule formation. Furthermore, inhibition of expression of osteoblast differentiation marker genes [such as type I collagen, alkaline phosphatase, and osteocalcin (OC)] was accompanied by suppression of osteoblast-specific transcription factors, Cbfa1/Runx2 and osterix. Flow cytometric analyses, cell adhesion, and inhibition assays disclosed alphavbeta3 and alphavbeta5 as the principal integrins mediating the adhesion of osteoblastic cells to betaig-h3. The disruption of interactions between betaig-h3 and osteoblasts by a function-blocking antibody specific for alphavbeta3 but not for alphavbeta5 abolished the inhibitory effect of betaig-h3 on osteoblast differentiation. We suggest that these interacting integrins may play an important role in betaig-h3-mediated inhibition of osteoblast differentiation.
 ...
PMID:Beta ig-h3 mediates osteoblast adhesion and inhibits differentiation. 1578 Sep 49