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http://purl.uniprot.org/citations/27083399http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/27083399http://www.w3.org/2000/01/rdf-schema#comment"The Col1a2(+/G610C) knock-in mouse, models osteogenesis imperfecta in a large old order Amish family (OOA) with type IV OI, caused by a G-to-T transversion at nucleotide 2098, which alters the gly-610 codon in the triple-helical domain of the α2(I) chain of type I collagen. Mineral and matrix properties of the long bones and vertebrae of male Col1a2(+/G610C) and their wild-type controls (Col1a2(+/+)), were characterized to gain insight into the role of α2-chain collagen mutations in mineralization. Additionally, we examined the rescuability of the composition by sclerostin inhibition initiated by crossing Col1a2(+/G610C) with an LRP(+/A214V) high bone mass allele. At age 10-days, vertebrae and tibia showed few alterations by micro-CT or Fourier transform infrared imaging (FTIRI). At 2-months-of-age, Col1a2(+/G610C) tibias had 13% fewer secondary trabeculae than Col1a2(+/+), these were thinner (11%) and more widely spaced (20%) than those of Col1a2(+/+) mice. Vertebrae of Col1a2(+/G610C) mice at 2-months also had lower bone volume fraction (38%), trabecular number (13%), thickness (13%) and connectivity density (32%) compared to Col1(a2+/+). The cortical bone of Col1a2(+/G610C) tibias at 2-months had 3% higher tissue mineral density compared to Col1a2(+/+); Col1a2(+/G610C) vertebrae had lower cortical thickness (29%), bone area (37%) and polar moment of inertia (38%) relative to Col1a2(+/+). FTIRI analysis, which provides information on bone chemical composition at ~7μm-spatial resolution, showed tibias at 10-days did not differ between genotypes. Comparing identical bone types in Col1a2(+/G610C) to Col1a2(+/+) at 2-months-of-age, tibias showed higher mineral-to-matrix ratio in trabeculae (17%) and cortices (31%). and in vertebral cortices (28%). Collagen maturity was 42% higher at 10-days-of-age in Col1a2(+/G610C) vertebral trabeculae and in 2-month tibial cortices (12%), vertebral trabeculae (42%) and vertebral cortices (12%). Higher acid-phosphate substitution was noted in 10-day-old trabecular bone in vertebrae (31%) and in 2-month old trabecular bone in both tibia (31%) and vertebrae (4%). There was also a 16% lower carbonate-to-phosphate ratio in vertebral trabeculae and a correspondingly higher (22%) carbonate-to-phosphate ratio in 2month-old vertebral cortices. At age 3-months-of-age, male femurs with both a Col1a2(+/G610C) allele and a Lrp5 high bone mass allele (Lrp5+/A214V) showed an improvement in bone composition, presenting higher trabecular carbonate-to-phosphate ratio (18%) and lower trabecular and cortical acid-phosphate substitutions (8% and 18%, respectively). Together, these results indicate that mutant collagen α2(I) chain affects both bone quantity and composition, and the usefulness of this model for studies of potential OI therapies such as anti-sclerostin treatments."xsd:string
http://purl.uniprot.org/citations/27083399http://purl.org/dc/terms/identifier"doi:10.1016/j.bone.2016.04.011"xsd:string
http://purl.uniprot.org/citations/27083399http://purl.uniprot.org/core/author"Huang Y."xsd:string
http://purl.uniprot.org/citations/27083399http://purl.uniprot.org/core/author"Ma Y."xsd:string
http://purl.uniprot.org/citations/27083399http://purl.uniprot.org/core/author"Wang M."xsd:string
http://purl.uniprot.org/citations/27083399http://purl.uniprot.org/core/author"Warman M.L."xsd:string
http://purl.uniprot.org/citations/27083399http://purl.uniprot.org/core/author"Marini J.C."xsd:string
http://purl.uniprot.org/citations/27083399http://purl.uniprot.org/core/author"Imbert L."xsd:string
http://purl.uniprot.org/citations/27083399http://purl.uniprot.org/core/author"Barnes A.M."xsd:string
http://purl.uniprot.org/citations/27083399http://purl.uniprot.org/core/author"Boskey A.L."xsd:string
http://purl.uniprot.org/citations/27083399http://purl.uniprot.org/core/author"Jacobsen C.M."xsd:string
http://purl.uniprot.org/citations/27083399http://purl.uniprot.org/core/author"Spevak L."xsd:string
http://purl.uniprot.org/citations/27083399http://purl.uniprot.org/core/author"Lukashova L."xsd:string
http://purl.uniprot.org/citations/27083399http://purl.uniprot.org/core/author"Masci M."xsd:string
http://purl.uniprot.org/citations/27083399http://purl.uniprot.org/core/date"2016"xsd:gYear
http://purl.uniprot.org/citations/27083399http://purl.uniprot.org/core/name"Bone"xsd:string
http://purl.uniprot.org/citations/27083399http://purl.uniprot.org/core/pages"120-129"xsd:string
http://purl.uniprot.org/citations/27083399http://purl.uniprot.org/core/title"Bone mineral properties in growing Col1a2(+/G610C) mice, an animal model of osteogenesis imperfecta."xsd:string
http://purl.uniprot.org/citations/27083399http://purl.uniprot.org/core/volume"87"xsd:string
http://purl.uniprot.org/citations/27083399http://www.w3.org/2004/02/skos/core#exactMatchhttp://purl.uniprot.org/pubmed/27083399
http://purl.uniprot.org/citations/27083399http://xmlns.com/foaf/0.1/primaryTopicOfhttps://pubmed.ncbi.nlm.nih.gov/27083399
http://purl.uniprot.org/uniprot/#_E0CXI2-mappedCitation-27083399http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/27083399
http://purl.uniprot.org/uniprot/#_H3BIY3-mappedCitation-27083399http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/27083399
http://purl.uniprot.org/uniprot/#_H3BJH0-mappedCitation-27083399http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/27083399
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