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Hutchinson Gilford Progeria

Get the facts on Hutchinson Gilford Progeria treatment, diagnosis, staging, causes, types, symptoms. Information and current news about clinical trials and trial-related data, Hutchinson Gilford Progeria prevention, screening, research, statistics and other Hutchinson Gilford Progeria related topics. We answer all your qestions about Hutchinson Gilford Progeria.

Question: Why does hutchinson gilford progeria present from birth whereas werner syndrome only manifests in puberty?

Answer: Werner Syndrome is a very rare, autosomal recessive disorder characterized by premature aging. Werner's syndrome more closely resembles accelerated aging than any other segmental progeria. For this reason, Werner syndrome is often referred to as a progeroid syndrome, as it partly mimics the symptoms of Progeria. The defect is on a gene that codes DNA helicase and it is located on the short arm of the 8th chromosome. The disorder is directly caused by shorter-than-normal length telomere maintenance. As a result DNA replication is impaired, thus making the times DNA can multiply in cells to not be able to have as many populations as normal cells do. Thus the effect will be delayed until the DNA telomere problem shows itself. Hutchinson-Gilford Progeria Syndrome (HGPS) is a childhood disorder caused by mutations in one of the major architectural proteins of the cell nucleus. This effect will be seen immediately from birth - the structural problem is present from day 1.

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