Saigal S, Bhargava A
Department of Oral Pathology, Government Dental College, Raipur(Chhattisgarh), India.
Kathmandu Univ Med J (KUMJ). 2012 Jan-Mar;10(37):72-6. doi: 10.3126/kumj.v10i1.6919.
Our life span is genetically programmed and it is possible that a defect in produced proteins encoded by the longevity gene is a cause of aging. Progeria which is a rare, fatal genetic condition which affects between one in four million and one in eight million children of both sexes equally and characterized by premature and accelerated aging. The appearance and physiology of these children resembles to elderly people but they typically have life span to their mid teens. It is also known as the Hutchinson-Gilford syndrome, which was initially reported by Johnathan Hutchinson in 1886 and further described by Hastings Gilford in 1904. It is an autosomal recessive disorder, which means an individual has inherited a mutated gene from both parents. It is added to the expanding catalogue of laminopathies, diseases caused by mutations affecting nuclear lamina proteins known as lamin A (LMNA). In oral manifestation primary finding is micrognathia with delayed tooth eruption and incomplete formation of root of permanent tooth. Presently there are no known cures for this abnormality.
我们的寿命是由基因编程的，长寿基因编码产生的蛋白质缺陷可能是衰老的一个原因。早衰症是一种罕见的致命遗传疾病，男女发病率相同，约为四百万分之一到八百万分之一，其特征是过早和加速衰老。这些儿童的外貌和生理机能类似于老年人，但他们的寿命通常到十几岁。它也被称为哈钦森-吉尔福德综合征，最初由乔纳森·哈钦森于1886年报道，1904年由黑斯廷斯·吉尔福德进一步描述。这是一种常染色体隐性疾病，这意味着个体从父母双方都继承了一个突变基因。它被添加到不断扩大的核纤层蛋白病目录中，核纤层蛋白病是由影响称为核纤层蛋白A（LMNA）的核纤层蛋白的突变引起的疾病。在口腔表现方面，主要发现是小颌畸形，伴有牙齿萌出延迟和恒牙牙根形成不完全。目前，这种异常尚无已知的治愈方法。
