Male hormones in development and reproduction Flashcards
Case 1: A 17 y/o female presents to her pediatrician with complaint that she has not had a menstrual period yet. She reports breast development but scant axillary and pubic hair. On exam, she is noted to have a blind vaginal pouch with no evidence of a uterus or cervix.
46,XY.
- lack of cervix, utuers and tubes: AMH
What cells secrete AMH?
Sertoli cells of testes
what does AMH do?
causes regression of female mullerian structures
what promotes development of testes?
SRY gene causes transcription factor TDF (testis determining factor) to be expressed.
- SRY gene is located on Y chromosome
what promotes development of male external genitalia? what enzyme is required?
DHT (dihydrotestosterone)
- 5alpha- reductase-2
5-alpha reductase 2 deficincies
results in ambiguous or feminzed external genitalia with not internal female structures
what cells secrete testosterone
Leydig cells
- action stimulated by LH
Before fetal pituitary LH action is initiated, which hormone stimulates fetal Leydig cell production of T?
hCG
- Note: hCG can be substituted for LH in clinical attempts to stimulate spermatogenesis in oligospermic men due to its increased availability. hCG is structurally most similar to LH and binds LH receptors.
Androgen insensitivity?
- patient does not develop a penis, scrotum or prostate but is secreting testosterone and has no 5alpha reductase 2 deficiency
- DHT is required for external male genitalia and prostate
- DHT binds same androgen receptor as testosterone, but with great afinity
- if patient is missing androgen receptors then target tissues do not respond to DHT/T
- this is an X-linked recessive trait
Do you expect a patient with androgen insensitivity to have seminal vesicles, ejaculatory ducts, and vas deferens/epididymis associated with the testes?
No, testosterone action required for these Wolffian duct structures to develop
Defective androgen receptors
What is required for formation of prostate gland and scrotum/penis?
DHT!!!!
Testosterone is converted to DHT via 5 alpha reductase 2
What is required for formation of prostate gland and scrotum/penis?
DHT!!!!
Testosterone is converted to DHT via 5 alpha reductase 2
What is required for formation of epididymis, Vas deferens, ejaculatory duct and seminal vesicle?
Just testosterone, secreted from Leydig cells
What is required for formation of epididymis, Vas deferens, ejaculatory duct and seminal vesicle?
Just testosterone, secreted from Leydig cells
What is required for development of Sertoli cells and Leydig cells?
SRY gene –> TDF –> development of testes –> development of these cell types –> secrete AMH and testosterone
What is required for development of Sertoli cells and Leydig cells?
SRY gene –> TDF –> development of testes –> development of these cell types –> secrete AMH and testosterone
when is SRY made?
6-8 weeks
when is SRY made?
6-8 weeks
When is testosterone/AMH made?
8 weeks