Sporadic Premature Aging in a Japanese Monkey: A Primate Model for Progeria
نویسندگان
چکیده
In our institute, we have recently found a child Japanese monkey who is characterized by deep wrinkles of the skin and cataract of bilateral eyes. Numbers of analyses were performed to identify symptoms representing different aspects of aging. In this monkey, the cell cycle of fibroblasts at early passage was significantly extended as compared to a normal control. Moreover, both the appearance of senescent cells and the deficiency in DNA repair were observed. Also, pathological examination showed that this monkey has poikiloderma with superficial telangiectasia, and biochemical assay confirmed that levels of HbA1c and urinary hyaluronan were higher than those of other (child, adult, and aged) monkey groups. Of particular interest was that our MRI analysis revealed expansion of the cerebral sulci and lateral ventricles probably due to shrinkage of the cerebral cortex and the hippocampus. In addition, the conduction velocity of a peripheral sensory but not motor nerve was lower than in adult and child monkeys, and as low as in aged monkeys. However, we could not detect any individual-unique mutations of known genes responsible for major progeroid syndromes. The present results indicate that the monkey suffers from a kind of progeria that is not necessarily typical to human progeroid syndromes.
منابع مشابه
Report of a four-year-old boy with Progeria without total Alopecia
Hutchinson-Gilford progeria syndrome is an extremely rare condition with features of premature and accelerated aging. The pattern of inheritance is unclear, although autosomal dominant mutations have been proposed. The disease presentation is usually in infancy and early childhood with a characteristic phenotype of short stature, abnormal skin and nail, beaked nose, loss of subcutaneous f...
متن کاملA Case Report of Hutchinson-Gilford Progeria Syndrome
Hutchinson-Gilford Progeria Syndrome (HGPS), a rare genetic condition occurs one in every 8 million live births. HGPS is characterized by premature aging in various organs. The average survival rate of the affected patients is 13 years, and their most common causes of death are myocardial infarction and stroke. This disease occurs due to a gene mutation in the chromosomes of the patient. There ...
متن کاملHutchinson-Guilford progeria syndrome.
Progeria is a human disease model of accelerated ageing. The progeria syndrome is a rare genetic disorder, first reported in 1886 by Hutchinson and Guilford in England. The inheritance pattern, paternal age eVect, and lack of consanguinity argue that it is due to a sporadic dominant mutation. HutchinsonGuilford progeria syndrome (HGPS) is associated with several features of premature ageing—for...
متن کاملHutchinson-gilford Syndrome (progeria): a Review
Progeria, also known as the Hutchinson-Gilford syndrome, is an extremely rare condition that was initially reported by Jonathan Hutchinson in 1886 and further described by Hastings Gilford in 1904. Hutchinson Gilford progeria syndrome is a disorder characterized by premature aging of postnatal onset. The main clinical and radiological features include alopecia, thin skin hypoplasia of nails, lo...
متن کاملAging: Progeria and the Lamin Connection
The relationship between progerias--diseases that resemble premature aging--and the normal aging process has been a source of debate in the aging research community. A recent study finds that LMNA, a gene targeted for mutation in Hutchinson Gilford Progeria Syndrome, may control the onset of aging-associated decline in normal fibroblasts.
متن کامل