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Accumulation of mutant lamin A causes progressive changes in nuclear architecture in Hutchinson-Gilford progeria syndrome

✍ Scribed by Goldman, R. D.; Shumaker, D. K.; Erdos, M. R.; Eriksson, M.; Goldman, A. E.; Gordon, L. B.; Gruenbaum, Y.; Khuon, S.; Mendez, M.; Varga, R.; Collins, F. S.


Book ID
121756783
Publisher
National Academy of Sciences
Year
2004
Tongue
English
Weight
677 KB
Volume
101
Category
Article
ISSN
0027-8424

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Hutchinson-Gilford progeria syndrome (HGPS) is a rare premature aging disorder normally caused by a spontaneous heterozygous mutation in the LMNA gene that codes for the nuclear lamina protein lamin A. Several enzymes are involved in the processing of its precursor, prelamin A, to the mature lamin A