2025
Improvements in hearing loss with bone-targeted enzyme replacement therapy are associated with corrected hypomineralization and osteocyte properties of auditory ossicles in Enpp1-deficient mice
Ocokoljic A, Srivastava S, von Kroge S, Stabach P, Busse B, Weise K, Oheim R, Rolvien T, Braddock D. Improvements in hearing loss with bone-targeted enzyme replacement therapy are associated with corrected hypomineralization and osteocyte properties of auditory ossicles in Enpp1-deficient mice. Journal Of Bone And Mineral Research 2025, zjaf082. PMID: 40495691, DOI: 10.1093/jbmr/zjaf082.Peer-Reviewed Original ResearchEnzyme replacement therapyHearing lossReplacement therapyENPP1 deficiencyAuditory brainstem response thresholdsAutosomal recessive hypophosphatemic rickets type 2Markers of mineral metabolismArterial calcification of infancyBrainstem response thresholdsElevated hearing thresholdsPrevent hearing lossWild-type miceImpairment of hearingAuditory ossiclesHearing thresholdsHearing functionHearing deficitsArtery calcificationDose-dependentlyMiddle earMouse modelOssicular chainBone propertiesMineral metabolismEffective treatment
2023
Lead induced differences in bone properties in osteocalcin +/+ and −/− female mice
Yildirim G, Budell W, Berezovska O, Yagerman S, Maliath S, Mastrokostas P, Tommasini S, Dowd T. Lead induced differences in bone properties in osteocalcin +/+ and −/− female mice. Bone Reports 2023, 18: 101672. PMID: 37064000, PMCID: PMC10090701, DOI: 10.1016/j.bonr.2023.101672.Peer-Reviewed Original ResearchBone strengthMineral/matrixBone remodelingHigher blood lead levelsSerum turnover markersLow lead exposureMajor health problemBlood lead levelsRole of osteocalcinBV/TVBone mineral propertiesTurnover markersFracture riskFemale miceLead exposureHealth problemsEffects of leadLead levelsBone formationCollagen maturityCross-sectional areaOsteocalcinLead toxicityBone propertiesBiomechanical measurements
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