progeria - significado y definición. Qué es progeria
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Qué (quién) es progeria - definición

GENETIC DISORDER THAT CAUSES EARLY AGING
Hutchinson Gilford Progeria Syndrome; Hutchinson–Gilford syndrome; Hutchinson Gilford Syndrom; Hutchinson-Gilford Syndrome; Projeria; Hutchinson-Gilford Progeria syndrome; Hutchinson-Gilford progeria syndrome; Hutchinson-Gilford; Hutchinson-Gilford Progeria Syndrome; Hutchinson–Gilford progeria syndrome; Hutchinson-Gilford syndrome; Pre Mature Old-Age; Hutchinson Gilford syndrome; Progeria syndrome; Progeria infantilis; Huchinson-Gilford Progeria; Hutchinson–Gilford–Progeria syndrome; Hutchinson-Gilford progeria; Hutchinson-Gilford Progeria; Progeria of childhood; Hutchinson-Gilford-Progeria syndrome; Hutchinson Gilford progeria syndrome; Progeric; Huchinson–Guilford progeria; Huchinson–Gilford progeria; Huchinson-Guilford progeria; Huchinson-Gilford progeria; Joseph syndrome
  • Ultrastructural analysis of the nuclear envelope in fibroblasts from a subject with HGPS. Low magnification transmission electron microscopic image of a passage 10 PT001 nucleus showed several herniations (a). Two higher-magnification images of the same nucleus at sites of blebs (b and c) showed a close apposition of the chromatin to the nuclear envelope. In a, b, and c, the nucleus is to the left. Scale bars correspond to 2 μm in panel a, and 500 nm in panels b and c.

progeria         
[pr?(?)'d???r??]
¦ noun Medicine a rare syndrome in children characterized by physical symptoms suggestive of premature old age.
Origin
early 20th cent.: mod. L., from Gk progeros 'prematurely old'.
Progeria Research Foundation         
ORGANIZATION
The Progeria Research Foundation (PRF) is a non-profit organization dedicated to developing treatments and, ultimately, a cure for progeria, a congenital disorder. The Foundation was established in 1999 by the family and friends of Sam Berns, a child with progeria, including Dr.
Werner syndrome         
LETHAL AUTOSOMAL RECESSIVE DISORDER
Werner's syndrome; Adult premature aging syndrome; Adult progeria; Werners Syndrome; Werners syndrome; Werner’s Syndrome; Atypical Werner syndrome; Werner's Syndrome; Werners; Werner's; Adult Progeria
Werner syndrome (WS) or Werner's syndrome, also known as "adult progeria",James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology.

Wikipedia

Progeria

Progeria is a specific type of progeroid syndrome, also known as Hutchinson–Gilford syndrome. A single gene mutation is responsible for progeria. The gene, known as lamin A (LMNA), makes a protein necessary for holding the nucleus of the cell together. When this gene gets mutated, an abnormal form of lamin A protein called progerin is produced. Progeroid syndromes are a group of diseases that causes individuals to age faster than usual, leading to them appearing older than they actually are. Patients born with progeria typically live to an age of mid-teens to early twenties.

Severe cardiovascular complications usually develop by puberty, resulting in death.

Ejemplos de uso de progeria
1. Premature ageing, or Hutchison–Gilford Progeria Syndrome (progeria), is obvious in the appearance of a child before it is a year old.
2. Zhou said the team came by their findings after comparing skin cells taken from two progeria sufferers, normal humans, progeria mice and normal mice.
3. While damaged DNA was quickly repaired in the healthy human and mice cell samples, the samples taken from the progeria humans and mice had difficulty repairing damaged DNA.
4. In 2003, a team of scientists in the United States found that progeria was caused by mutation in a protein called Lamin A, which lines the nucleus in human cells.
5. "Mutation in this protein (Lamin A) can cause defects in repair and thus lead to progeria," Zhou, a research assistant professor with the biochemistry department at the University of Hong Kong, said in an interview.