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It is caused by mutations in the LMNA lamin A protein gene on chromosome 1 ; the mutated form of lamin A is commonly known as progerin.

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These results have been observed only in vitro and are not the results of any clinical trial, although it is believed that the treatment might benefit HGPS patients.

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Although the term progeria applies strictly speaking to all diseases characterized by premature aging symptoms, and is often used as such, it is often applied specifically in reference to Hutchinson—Gilford progeria syndrome HGPS.

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