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PROGERIA AND WERNER'S SYNDROME AS MODELS FOR THE STUDY OF NORMAL HUMAN AGING *
Author(s) -
REICHEL WILLIAM,
GARCIABUNUEL RAFAEL,
DILALLO JOSEPH
Publication year - 1971
Publication title -
journal of the american geriatrics society
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.992
H-Index - 232
eISSN - 1532-5415
pISSN - 0002-8614
DOI - 10.1111/j.1532-5415.1971.tb03230.x
Subject(s) - progeria , medicine , connective tissue , premature aging , disease , genetic disorder , elastin , pathology , genetics , biology , physiology , gene
A bstract : Progeria and perhaps Werner's syndrome represent important models for the study of normal human aging. A case of progeria is presented. If a connective‐tissue disorder is the basis of progeria, this could account for the resemblance to human aging changes in skin, blood vessels and myocardium. An alternate hypothesis is that progeria is not merely a connective‐tissue disorder that resembles normal aging, but is aging prematurely expressed. A disorder in the normal genetic regulatory mechanism that controls the onset of aging could cause a progeric condition with the associated connective‐tissue changes and other aging changes prematurely seen. It is vital to determine which of these two mechanisms is the cause of this disease. Such an understanding may provide great insights into the normal biological mechanisms of development and aging.