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耳硬化症的分子生物学

Molecular biology of otosclerosis.

作者信息

McKenna Michael J, Kristiansen Arthur G

机构信息

Department of Otology and Laryngology, Harvard Medical School, Boston, Mass., USA.

出版信息

Adv Otorhinolaryngol. 2007;65:68-74. doi: 10.1159/000098674.

Abstract

Otosclerosis is a bone disease of the human otic capsule, which is among the most common causes of acquired hearing loss. The pathologic process is characterized by a wave of abnormal bone remodeling in specific sites of predilection within the endochondral layer of the temporal bone. Although the cause of otosclerosis remains uncertain, there is a clear genetic predisposition with half of all cases occurring in families with more than one affected member. There is also compelling evidence that measles virus may play a role in some cases. Ultimately, how genetic factors and viral infection result in otosclerosis must be explained by effects on the molecular factors that control bone remodeling.

摘要

耳硬化症是一种人类听骨囊的骨疾病,是后天性听力损失最常见的原因之一。病理过程的特征是颞骨软骨内层面特定偏好部位出现异常骨重塑浪潮。虽然耳硬化症的病因尚不确定,但存在明显的遗传易感性,所有病例中有一半发生在有多名受影响成员的家庭中。也有令人信服的证据表明麻疹病毒在某些病例中可能起作用。最终,遗传因素和病毒感染如何导致耳硬化症必须通过对控制骨重塑的分子因素的影响来解释。

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