Mouse models for Down syndrome-associated developmental cognitive disabilities.
نویسندگان
چکیده
Down syndrome (DS) is mainly caused by the presence of an extra copy of human chromosome 21 (Hsa21) and is a leading genetic cause for developmental cognitive disabilities in humans. The mouse is a premier model organism for DS because the regions on Hsa21 are syntenically conserved with three regions in the mouse genome, which are located on mouse chromosome 10 (Mmu10), Mmu16 and Mmu17. With the advance of chromosomal manipulation technologies, new mouse mutants have been generated to mimic DS at both the genotypic and phenotypic levels. Further mouse-based molecular genetic studies in the future may lead to the unraveling of the mechanisms underlying DS-associated developmental cognitive disabilities, which would lay the groundwork for developing effective treatments for this phenotypic manifestation. In this review, we will discuss recent progress and future challenges in modeling DS-associated developmental cognitive disability in mice with an emphasis on hippocampus-related phenotypes.
منابع مشابه
Aging and intellectual disability: insights from mouse models of Down syndrome.
Down syndrome (DS) is one of many causes of intellectual disability (ID), others including but not limited to, fetal alcohol syndrome, Fragile X syndrome, Rett syndrome, Williams syndrome, hypoxia, and infection. Down syndrome is characterized by a number of neurobiological problems resulting in learning and memory deficits and early onset Alzheimer's disease. The cognitive impairment in people...
متن کاملThe Down syndrome critical region regulates retinogeniculate refinement.
Down syndrome (DS) is a developmental disorder caused by a third chromosome 21 in humans (Trisomy 21), leading to neurological deficits and cognitive impairment. Studies in mouse models of DS suggest that cognitive deficits in the adult are associated with deficits in synaptic learning and memory mechanisms, but it is unclear whether alterations in the early wiring and refinement of neuronal ci...
متن کاملIndividual and environmental characteristics associated with cognitive development in Down syndrome: a longitudinal study.
BACKGROUND Associations among cognitive development and intrapersonal and environmental characteristics were investigated for 89 longitudinal study participants with Down syndrome to understand developmental patterns associated with cognitive strengths and weaknesses. MATERIALS AND METHODS Subtest scores of the Stanford-Binet IV collected between ages 4-30 years were analysed in multilevel mo...
متن کاملNeurogenetic Disorders of Down Syndrome and Potential Pharmacotherapies for Mental Retardation
Background: Trisomy of human chromosome 21 is the most frequent genetic cause of mental retardation or intellectual disability and others phenotypes, including developmental defects, dysmorphic features and cognitive impairments collectively known as Down syndrome. Mainly a consequence of developmental and functional brain alterations, the mental retardation is the most invariable and invalidat...
متن کاملMouse Models of Down Syndrome as a Tool to Unravel the Causes of Mental Disabilities
Down syndrome (DS) is the most common genetic cause of mental disability. Based on the homology of Hsa21 and the murine chromosomes Mmu16, Mmu17 and Mmu10, several mouse models of DS have been developed. The most commonly used model, the Ts65Dn mouse, has been widely used to investigate the neural mechanisms underlying the mental disabilities seen in DS individuals. A wide array of neuromorphol...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Developmental neuroscience
دوره 33 5 شماره
صفحات -
تاریخ انتشار 2011