scholarly journals Correction: Global deletion of optineurin results in altered type I IFN signaling and abnormal bone remodeling in a model of Paget’s disease

Author(s):  
Sing-Wai Wong ◽  
Bo-Wen Huang ◽  
Xiangxiang Hu ◽  
Eui Ho Kim ◽  
Joseph P. Kolb ◽  
...  
2019 ◽  
Vol 27 (1) ◽  
pp. 71-84 ◽  
Author(s):  
Sing-Wai Wong ◽  
Bo-Wen Huang ◽  
Xiangxiang Hu ◽  
Eui Ho Kim ◽  
Joseph P. Kolb ◽  
...  

2017 ◽  
Vol 22 (4-5) ◽  
pp. 218-225 ◽  
Author(s):  
Takefumi Kamakura ◽  
Joseph B. Nadol Jr.

Bone remodeling within the otic capsule has been reported to be inhibited especially at or near the cochlea, except under some pathological conditions such as otosclerosis, Paget's disease, or mastoiditis, when bone remodeling can occur. Microcavitations found in periosteal and endosteal layers of human temporal bone specimens without otosclerosis, Paget's disease, or inflammation as reported in the current study are consistent with osteoclastic bone resorption. Thirty-three temporal bones from 33 patients were prepared for light microscopy and classified into 4 groups: histologically proven dehiscence of the superior semicircular canal (SSCD) (n = 3, group 1), age 20 years or younger (n = 10, group 2), age 90 years or older and with otosclerosis (n = 10, group 3), and age 90 years or older without otosclerosis (n = 10, group 4). Microcavitation was seen at 7 anatomic locations in the temporal bone in all 4 groups, but not in the cochlea or vestibule. Microcavitation within the temporal bone is likely due to osteoclastic activity, and it is seen in both young and old patients, patients with and without otosclerosis, and in cases with SSCD.


2000 ◽  
Vol 345 (3) ◽  
pp. 473-480 ◽  
Author(s):  
Paul A. C. CLOOS ◽  
Christian FLEDELIUS

Fragments of the α1 C-terminal telopeptide of type I collagen containing the sequence AHDGGR1209-1214 (CTx) can be measured in urine as an index of bone resorption. We report here that these molecules undergo racemization and isomerization of Asp1211in vitro and in vivo, generating a mixture of four isomers: the native peptide form (αL), an isomerized form containing a β-Asp bond (βL), a racemized form containing a D-Asp residue (αD) and an isomerized/racemized form (βD). To study these reactions at this specific site in collagen, we have employed four immunoassays, each specific for one of the isoforms, and developed HPLC methods for their separation. The kinetics of these reactions were studied in vitro under physiological conditions by incubation of synthetic AHDGGR hexapeptide or mineralized bone collagen. Reactions were found to be strongly shifted towards the β-Asp forms and slightly in favour of the D-enantiomeric forms. CTx isomers were measured in human urine and in enzymic digests of bovine bone collagen. The results indicated that the extent of racemization and isomerization were correlated with the age and turnover of collagen. The ratios between the native and age-related forms of CTx were elevated in urine from patients with Paget's disease or osteoporosis as compared with that from healthy adults. The αL/αD CTx ratio had the highest discriminatory power (T-score = 23.2; P < 0.0001 and T-score = 1.5; P < 0.0001 for Paget's disease and osteoporosis respectively). In conclusion, these findings indicate that an assessment of CTx ratios in urine may provide an estimate of bone turnover, aiding in the diagnosis of metabolic bone diseases.


1980 ◽  
Vol 163 (4) ◽  
pp. 547-552 ◽  
Author(s):  
H. S. Cheung ◽  
F. R. Singer ◽  
B. Mills ◽  
M. E. Nimni

2014 ◽  
Vol 1 (1) ◽  
pp. 36
Author(s):  
R. Dineshwarran

Paget's disease is a disorder of bone metabolism causing excessive bone remodeling which is usually seen in elderly patients. A typical case of maxillary involvement along with the craniofacial clinical and radiographical findings is presented.


Author(s):  
Maria R. Bonnin ◽  
Carmen Moragues ◽  
Juan M. Nolla ◽  
Francisco J. Lirón ◽  
Daniel Roig-Escofet ◽  
...  

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