Step I kallmann syndrome

Started by DrPettans
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DrPettans

Full Member
7+ Year Member
Kallmann sd is a defective cell/neuron migration that involves low GnRH and anosmia. It's X-linked.
This low GnRH implies a tertiary hypogonadism (hypogonadotropic hypogonadism), I understand that. But:

If GnRH pulses is what stimulates FSH and LH, and then:
- FSH stimulates Sertoli cells to secrete MIF so Mullerian ducts won't develop as Fallopian tubes + uterus + upper vagina.
- LH stimulates Leydig cells to secrete Testosterone which makes the mesonephric ducts (Wolfferian ducts) to develop as male internal genitalia.
- Testosterone is converted into DHT by 5alpha reductase and then DHT makes the external genitalia to develop.

My question is: what appearance would an XY human being with this deffect have?? testes + female internal genitalia (uterus + fallopian tubes + vagina) + female external genitalia (labia and clit) ?
And also: is it possible on an XX fetus? If so, what would happen in terms of sex development?

If DHT is responsible for male external genitalia development (scrotum and penis), what is responsible for female external genitalia development (clit and labia)?