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Chapter 3: Problem 15

Describe two ways that children with Hutchinson-Gilford progeria syndrome age rapidly.

Short Answer

Expert verified
Children with Hutchinson-Gilford Progeria Syndrome age rapidly primarily due to the production of an abnormal protein - Progerin, which causes deformations in cellular structure leading to an accelerated aging process. Physically, these children exhibit symptoms more common in the elderly such as growth failure, loss of body fat and hair, skin aging, atherosclerosis and more, which all are resultants of the internal cellular aging.

Step by step solution

01

Understanding Hutchinson-Gilford progeria syndrome

Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare genetic disorder that causes rapid aging in children. It is most often caused by a mutation in the LMNA gene, which codes for the Lamin A protein. The mutation leads to the production of an abnormal version of the Lamin A protein, known as Progerin.
02

Explaining the role of Progerin at the Cellular level

This abnormal Progerin protein, due to its more solid form, tightly binds to the nuclear rim resulting in an irreversible structural deformation of the cell nucleus. This alters the normal functioning of cells, which becomes a consistent feature of aging, leading to the rapid onset of aging symptoms observed in children with HGPS.
03

Detailing the Physical manifestations

Progeria also impacts the child physically with symptoms that typically include growth failure, loss of body fat and hair, aged-looking skin, stiffness of joints, hip dislocation, generalized atherosclerosis, cardiovascular disease and stroke. These symptoms, usually absent in children, are characteristics of normal aging process but appear at a very early age in children with Progeria.

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