How is Progeria inherited

Progeria Type I (HGPS): causes

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Has progeria type I (HGPS) genetic causes. The reason is a change in the genome, namely in the so-called lamin A / C gene. With the help of this gene, the body produces two specific proteins, Lamin A and Lamin C, which stabilize the cell body envelope and are involved in cell division. In progeria, a "letter" - a so-called base - is swapped in the genetic code of the lamin gene. Instead of a functional protein Lamin A, the organism in children with progeria only produces one shortened version. This change weakens the envelope of the cell nucleus and disrupts processes such as cell division; it may also cause the genetic information to be broken down prematurely.

HGPS is usually used not inherited. There are almost exclusively isolated cases of the disease known.

Scientists believe that type I progeria is a consequence random changes in the genome (mutations) acts. The parents of the affected children then - in contrast to their child - do not show any abnormalities in the gene for lamin A / C. Why the lamin gene mutates in individual cases cannot be clarified. Such Spontaneous mutations are random events. Only in very rare cases of progeria type I do healthy parents already carry a mutation for Lamin A and pass the defective genetic material on to their child.