LARON SYNDROME: DIFFERENT ASPECTS OF INSULIN-LIKE GROWTH FACTOR-1 DEFICIENCY

Abstract

Annotation: Laron syndrome (Laron-type dwarfism) is a rare endocrine genetic disorder with an autosomal recessive inheritance pattern caused by a variety of mutations in the growth hormone receptor. Typical manifestations include dwarfism, «doll» face and obesity. Currently, the number of patients in the world is about 350. It is noteworthy that these people almost never suffer from cancer, show a delayed age-related decline in cognitive abilities and some of them are quite resistant to type 2 diabetes. Moreover, animals with artificially induced «Laron syndrome» have a markedly increased lifespan. All these phenomena are explained by the fact that with this disease in the blood there is a very low level of insulin-like growth factor-1 — a compound that is normally secreted by cells in response to the effect of growth hormone and mediates its action in tissues. These observations suggest that the study of Laron syndrome may allow one to get closer to understanding the endocrine mechanisms of cancer and a number of other pathological processes, and, in addition, throw light on some of the molecular genetic foundations of aging and longevity. This article provides information on some of the already discovered interesting consequences of congenital deficiency of insulin-like growth factor-1, further study of which seems to be very promising.