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Progeria Syndrome

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Synonyms

Progeria or Hutchinson-Gilford Syndrome

Definition

Progeria syndrome is a very rare, genetic syndrome that is characterized by greatly exaggerated premature aging; an individual with progeria syndrome generally succumbs to heart attack or stroke by age 13 [6]. In more detail, progeria is a sporadically occurring, autosomal disorder meaning that only one copy of a gene is necessary for the condition [3]. To understand the basis for progeria’s occurrence, a brief summary of genetic mechanism is needed. The recipe for generating and following competent genetic instructions is relatively simple to understand. The molecule of genetic inheritance, DNA, is a string of DNA nucleotides which accords a nearly identical RNA nucleotide string. The RNA nucleotide string is itself transformed or translated into an a chain of amino acids that can become folded into a protein [2]. The alphabetical code or nucleotides for DNA and RNA are differentiated from one or the other by their chemical...

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  • DOI: 10.1007/978-0-387-79061-9_2264
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References

  1. Carlson, N. R. (2007). Physiology of behavior (9th ed.). New York: Pearson.

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  2. Chamary, J. V., & Hurst, L. D. (2009). The price of silent mutations. Scientific American, 300, 46–53.

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  3. Chambers, D. (2008). Autosomal dominant. In Medline plus medical encyclopedia. Retrieved June 9, 2009, from http://www.nlm.nih.gov/MEDLINEPLUS/ency/article/002049.htm

  4. Foisner, R. (2001). Inner nuclear membrane proteins and the nuclear lamina. Journal of Cell Science, 114, 3791–3792. Retrieved June 8, 2009, from http://jcs.biologists.org/cgi/content/full/114/21/3791

  5. Korf, B. (2008). Hutchinson-Gilford progeria syndrome, aging and the nuclear lamina. New England Journal of Medicine, 338(6), 552–555.

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  6. Meredith, M. A., Gordon, L. B., Clauss, S., Sachdev, V., Smith, A. C. M., Berry, M. B., et al. (2008). Phenotype and course of Hutchinson-Gilford progeria syndrome. New England Journal of Medicine, 338(6), 592–604.

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© 2011 Springer Science+Business Media, LLC

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Phelps, B.I. (2011). Progeria Syndrome. In: Goldstein, S., Naglieri, J.A. (eds) Encyclopedia of Child Behavior and Development. Springer, Boston, MA. https://doi.org/10.1007/978-0-387-79061-9_2264

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