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Prospective signs of cleidocranial dysplasia in Cebpb deficiency
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نویسنده
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huang b. ,takahashi k. ,jennings e.a. ,pumtang-on p. ,kiso h. ,togo y. ,saito k. ,sugai m. ,akira s. ,shimizu a. ,bessho k.
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منبع
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journal of biomedical science - 2014 - دوره : 21 - شماره : 1
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چکیده
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Background: although runt-related transcription factor 2 (runx2) has been considered a determinant of cleidocranial dysplasia (ccd),some ccd patients were free of runx2 mutations. ccaat/enhancer-binding protein beta (cebpb) is a key factor of runx2 expression and our previous study has reported two ccd signs including hyperdontia and elongated coronoid process of the mandible in cebpb deficient mice. following that,this work aimed to conduct a case-control study of thoracic,zygomatic and masticatory muscular morphology to propose an association between musculoskeletal phenotypes and deficiency of cebpb,using a sample of cebpb,cebpb and cebpb adult mice. somatic skeletons and skulls of mice were inspected with soft x-rays and micro-computed tomography (μct),respectively. zygomatic inclination was assessed using methods of coordinate geometry and trigonometric function on anatomic landmarks identified with μct. masseter and temporal muscles were collected and weighed. expression of cebpb was examined with a reverse transcriptase polymerase chain reaction (rt-pcr) technique. results: cebpb mice displayed hypoplastic clavicles,a narrow thoracic cage,and a downward tilted zygomatic arch (p < 0.001). although cebpb mice did not show the phenotypes above (p = 0.357),a larger mass percentage of temporal muscles over masseter muscles was seen in cebpb littermates (p = 0.012). the mrna expression of cebpb was detected in the clavicle,the zygoma,the temporal muscle and the masseter muscle,respectively. conclusions: prospective signs of ccd were identified in mice with cebpb deficiency. these could provide an additional aetiological factor of ccd. succeeding investigation into interactions among cebpb,runx2 and musculoskeletal development is indicated. © 2014 huang et al.; licensee biomed central ltd.
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کلیدواژه
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Cebpb; Clavicle; Cleidocranial dysplasia; Masseter; Temporal muscle; Thoracic cage; Zygomatic arch
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آدرس
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school of medicine and dentistry,james cook university,cairns,australia,department of oral and maxillofacial surgery,graduate school of medicine,kyoto university,shogoin-kawahara-cho 54,sakyo-ku,kyoto 606-8507, Japan, department of oral and maxillofacial surgery,graduate school of medicine,kyoto university,shogoin-kawahara-cho 54,sakyo-ku,kyoto 606-8507, Japan, school of medicine and dentistry,james cook university,cairns, Australia, setthasiri animal hospital,bangkok, Thailand, department of oral and maxillofacial surgery,graduate school of medicine,kyoto university,shogoin-kawahara-cho 54,sakyo-ku,kyoto 606-8507, Japan, department of oral and maxillofacial surgery,graduate school of medicine,kyoto university,shogoin-kawahara-cho 54,sakyo-ku,kyoto 606-8507, Japan, department of oral and maxillofacial surgery,graduate school of medicine,kyoto university,shogoin-kawahara-cho 54,sakyo-ku,kyoto 606-8507, Japan, translational research center,kyoto university hospital,kyoto university,kyoto, Japan, laboratory of host defense,world premier international immunology,frontier research center,osaka university,osaka, Japan, translational research center,kyoto university hospital,kyoto university,kyoto, Japan, department of oral and maxillofacial surgery,graduate school of medicine,kyoto university,shogoin-kawahara-cho 54,sakyo-ku,kyoto 606-8507, Japan
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