Abstract
Studying of the genetic mutant is a time-honored approach to understanding the molecular mechanism behind a disease. This method has also been widely used in the study of aging. As aging has been shown to be associated with genetic factors, the study of premature aging mutants allows us to understand what goes wrong in molecular level when we age. This chapter reviews an extremely rare and fatal premature aging disease, Hutchinson–Gilford progeria syndrome (HGPS). HGPS is caused by mutations in LMNA gene, which results in the production of truncated lamin A known as progerin. HGPS features symptoms resembling aspects of physiological aging, such as alopecia, osteoporosis, cardiovascular diseases, etc. These features make HGPS a good disease model for the study of aging. This chapter will firstly describe the genetic and clinical background of HGPS and the current therapeutic schemes for HGPS patients. Secondly, this chapter will introduce mouse models developed to mimic features of HGPS, which are used extensively in the study of premature aging and physiological aging. Thirdly, the proposed molecular mechanisms behind HGPS will be discussed. Lastly, we will discuss the relationship between HGPS and physiological aging.
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