Matches in SemOpenAlex for { <https://semopenalex.org/work/W1990019150> ?p ?o ?g. }
- W1990019150 endingPage "2026" @default.
- W1990019150 startingPage "2015" @default.
- W1990019150 abstract "Hypophosphatasia is an inborn error of metabolism characterized by deficient activity of the tissue-nonspecific isoenzyme of alkaline phosphatase (TNSALP) and skeletal disease due to impaired mineralization of cartilage and bone matrix. We investigated two independently generated TNSALP gene knock-out mouse strains as potential models for hypophosphatasia. Homozygous mice (-/-) had < 1% of wild-type plasma TNSALP activity; heterozygotes had the predicted mean of approximately 50%. Phosphoethanolamine, inorganic pyrophosphate, and pyridoxal 5'-phosphate are putative natural substrates for TNSALP and all were increased endogenously in the knock-out mice. Skeletal disease first appeared radiographically at approximately 10 days of age and featured worsening rachitic changes, osteopenia, and fracture. Histologic studies revealed developmental arrest of chondrocyte differentiation in epiphyses and in growth plates with diminished or absent hypertrophic zones. Progressive osteoidosis from defective skeletal matrix mineralization was noted but not associated with features of secondary hyperparathyroidism. Plasma and urine calcium and phosphate levels were unremarkable. Our findings demonstrate that TNSALP knock-out mice are a good model for the infantile form of hypophosphatasia and provide compelling evidence for an important role for TNSALP in postnatal development and mineralization of the murine skeleton." @default.
- W1990019150 created "2016-06-24" @default.
- W1990019150 creator A5016846659 @default.
- W1990019150 creator A5019660823 @default.
- W1990019150 creator A5023255203 @default.
- W1990019150 creator A5030109852 @default.
- W1990019150 creator A5035967512 @default.
- W1990019150 creator A5036775753 @default.
- W1990019150 creator A5046642788 @default.
- W1990019150 creator A5070431799 @default.
- W1990019150 creator A5070552820 @default.
- W1990019150 creator A5079706379 @default.
- W1990019150 creator A5085434785 @default.
- W1990019150 date "1999-12-01" @default.
- W1990019150 modified "2023-10-17" @default.
- W1990019150 title "Alkaline Phosphatase Knock‐Out Mice Recapitulate the Metabolic and Skeletal Defects of Infantile Hypophosphatasia" @default.
- W1990019150 cites W152506992 @default.
- W1990019150 cites W1588199014 @default.
- W1990019150 cites W1599513095 @default.
- W1990019150 cites W1916253178 @default.
- W1990019150 cites W1968638936 @default.
- W1990019150 cites W1968930811 @default.
- W1990019150 cites W1969012832 @default.
- W1990019150 cites W1980519659 @default.
- W1990019150 cites W1989031800 @default.
- W1990019150 cites W1994460887 @default.
- W1990019150 cites W2002273560 @default.
- W1990019150 cites W2008544782 @default.
- W1990019150 cites W2020312178 @default.
- W1990019150 cites W2054829715 @default.
- W1990019150 cites W2056825523 @default.
- W1990019150 cites W2064113953 @default.
- W1990019150 cites W2067376187 @default.
- W1990019150 cites W2075528851 @default.
- W1990019150 cites W2082446278 @default.
- W1990019150 cites W2082920380 @default.
- W1990019150 cites W2085392426 @default.
- W1990019150 cites W2092762214 @default.
- W1990019150 cites W2109430357 @default.
- W1990019150 cites W2116365881 @default.
- W1990019150 cites W2145182226 @default.
- W1990019150 cites W2146093324 @default.
- W1990019150 cites W2152169709 @default.
- W1990019150 cites W2153435210 @default.
- W1990019150 cites W2157925286 @default.
- W1990019150 cites W2170455029 @default.
- W1990019150 cites W322800605 @default.
- W1990019150 cites W4211112402 @default.
- W1990019150 cites W4248820996 @default.
- W1990019150 doi "https://doi.org/10.1359/jbmr.1999.14.12.2015" @default.
- W1990019150 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/3049802" @default.
- W1990019150 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/10620060" @default.
- W1990019150 hasPublicationYear "1999" @default.
- W1990019150 type Work @default.
- W1990019150 sameAs 1990019150 @default.
- W1990019150 citedByCount "350" @default.
- W1990019150 countsByYear W19900191502012 @default.
- W1990019150 countsByYear W19900191502013 @default.
- W1990019150 countsByYear W19900191502014 @default.
- W1990019150 countsByYear W19900191502015 @default.
- W1990019150 countsByYear W19900191502016 @default.
- W1990019150 countsByYear W19900191502017 @default.
- W1990019150 countsByYear W19900191502018 @default.
- W1990019150 countsByYear W19900191502019 @default.
- W1990019150 countsByYear W19900191502020 @default.
- W1990019150 countsByYear W19900191502021 @default.
- W1990019150 countsByYear W19900191502022 @default.
- W1990019150 countsByYear W19900191502023 @default.
- W1990019150 crossrefType "journal-article" @default.
- W1990019150 hasAuthorship W1990019150A5016846659 @default.
- W1990019150 hasAuthorship W1990019150A5019660823 @default.
- W1990019150 hasAuthorship W1990019150A5023255203 @default.
- W1990019150 hasAuthorship W1990019150A5030109852 @default.
- W1990019150 hasAuthorship W1990019150A5035967512 @default.
- W1990019150 hasAuthorship W1990019150A5036775753 @default.
- W1990019150 hasAuthorship W1990019150A5046642788 @default.
- W1990019150 hasAuthorship W1990019150A5070431799 @default.
- W1990019150 hasAuthorship W1990019150A5070552820 @default.
- W1990019150 hasAuthorship W1990019150A5079706379 @default.
- W1990019150 hasAuthorship W1990019150A5085434785 @default.
- W1990019150 hasBestOaLocation W19900191502 @default.
- W1990019150 hasConcept C104317684 @default.
- W1990019150 hasConcept C105702510 @default.
- W1990019150 hasConcept C124490489 @default.
- W1990019150 hasConcept C126322002 @default.
- W1990019150 hasConcept C134018914 @default.
- W1990019150 hasConcept C135763542 @default.
- W1990019150 hasConcept C153471976 @default.
- W1990019150 hasConcept C160160445 @default.
- W1990019150 hasConcept C181199279 @default.
- W1990019150 hasConcept C2776489590 @default.
- W1990019150 hasConcept C2778471162 @default.
- W1990019150 hasConcept C2780550940 @default.
- W1990019150 hasConcept C2781403057 @default.
- W1990019150 hasConcept C55493867 @default.
- W1990019150 hasConcept C71924100 @default.
- W1990019150 hasConcept C86803240 @default.
- W1990019150 hasConceptScore W1990019150C104317684 @default.