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Image: An international team of researchers led by KAUST has identified new targets for treating the syndrome that causes premature aging in children
Progeria-like syndrome is a group of rare genetic disorders that cause signs of premature aging in children and young adults, such as Werner syndrome and Hutchinson-Guildford Progeria syndrome
This aging is characterized by a gradual loss of nuclear structure and an underlying tissue-specific genetic process, but the reasons behind this remain unclear
The target is called long-spaced nucleo-1 (L1) RNA — a family of repeats that contain about 17-20 percent of the mammalian genome, and whose function is largely unknown
Sequencing analysis conducted by KAUST and the U.
The researchers were able to block the expression of L1 RNA, reversing the aging process in cells taken from patients with Progeria-like syndrome and mice
Further research will need to determine whether other mechanisms, in parallel with SUV39H1 inhibition, may impair heterochromatin stability
"In other observations, our work establishes an important rule," said
Orlando said: "Given the similarities between Progeria-like syndrome and age-related diseases, targeting LINE-1 RNA may be an effective treatment for Progeria-like syndrome and other age-related diseases characterized by abnormal expression of LINE-1, such as neurodegeneration, metabolic and cardiovascular disease and cancer
essay
LINE-1 RNA causes heterochromatin erosion and is a target for amelioration of senescent phenotypes in progeroid syndromes
