What is testicular feminization syndrome

Under what factors in a person having a Y-chromosome, generated female appearance.

syndrome of testicular feminization (syndrome or androgen insensitivity) - a disease that occurs when the full or partial lack of tissue sensitivity to androgens.

This female phenotype (appearance) is formed by a person with normal male genotype (set of genes) and the presence of the X and Y chromosomes.And the appearance of these women can not even suspect they have the Y-chromosome.A case where this diagnosis was a girl in the 60s of the last century became Miss World.

Why is this happening

reason for this insensitivity of peripheral tissues to the hormone receptor is a congenital disorder caused by a mutation in the androgen receptor gene.This pathology is found in one of the 50-70 thousand. Newborn.This syndrome refers to a hereditary disease linked with X-linked recessive type.

Depending on the degree of androgen receptor insensitivity to distinguish the full form of testicular feminization (full androgen ins

ensitivity) and incomplete form (when the sensitivity is initially partially retained or partially reversed in adolescence).

Why newborns look like the girls in spite of male genotype

During embryonic gonads with testicular feminization syndrome in the seventh week of fetal development under the influence of Y-chromosomes are differentiated as full functioning testicles.In order to form the primary sexual characteristics of male type, need androgens.

Under the influence of two hormones (testosterone and dihydrotestosterone) are formed penis, scrotum, urethra, and prostate gland (prostate).However, due to a defect of the gene tissue of patients with androgen insensitivity, but the sensitivity to estrogen is stored.

This leads to a natural development of the female phenotype.Female internal reproductive organs (fallopian tubes, uterus and upper third of the vagina) in these patients are not available, because it is synthesized in the testes factor that inhibits their development of Müllerian ducts.

Testicles can be located in the abdomen, and in the inguinal canal or labia lips.When intraperitoneal localization testicular very high probability of malignant transformation, which increases with age.

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