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69 Cards in this Set

  • Front
  • Back
When and how does sex determination occur?
sex determination is established by the sex chromosomes of the genome at fertilization
What two sex differnetiation factors must be present in order to from a proper male
-presence of androgens
-presence of MIS/MIF
What is the function of MIS/MIF
MIS/MIF if a glycoprotein secreted by the testis until age 10. The receptors are located on the paramesonephric (mullerian) ducts and binding to the receptor causes the ducts to apoptose. The ducts are the precursors of the oviducts, uterus, and part of the vagina so presence of MIS is necessary for proper male development
Describe the result of sex differntiation in the brain
Determines how GnRH will be released. Males release it in a pulsitile manner while females are cyclic. In males, the differentiation of the brain is caused by a surge of testosterone at 6 months after birth
When and where do the gonad primorida form
Form during the 4th week in the ventromedial portion of the nephrogenic ridge as a proliferation of the overlying coelomic epithelium and ridge mesenchyme. This becomes the urogenital ridge
What three cell types contribute to the formation of the gonad
1. germinal cells
2. supporting cells
3. interstitial cells
Describe the origin and migration of the primordial germ cells which will form the stem cells for gamete production
Originate extragonadally in the yolk sac (from epiblast?) during week 3. During weeks 4-5 they migrate to the gonad within the dorsal mesentery of the hindgut where they continue to divide.
Describe the formation of the supproting cells (males= sertoli, females= granulosa)
originate from cord lke proliferations of the coelomic epitheium overlying the gonad primordium. They play a major role in organizing the gonad
Describe the formation o fthe intersitial cells (males=leydig, females= theca)
Originate from mesonephric mesenchyme. They synthesize and secrete steriod hormones.
What are the two attachesments of the primitive gonad
Both are formed crom surronding mesenchymal tissues
1. cranial= suspensorly ligament (vascualr supply develops here)
2. Caudally= gubernaclum (attaches gonad to ventral body wall)
What is the origin of the mesonephric/ wolffian ducts
form during week 4 within the neprhogenic ridge as part of the mesonephros
What is the origin of the paramesonephric ducts
Devleop during week 6 as a response to signals from the mesonephric ducts. They being as an invagination of the ceolomic epithelium covering the lateral aspect of the UG ridge
Describe the formation of the uterovaginal primordium
A fusion of the inferior 1/3 of the paramesonephric ducts
Where do the paramesonephric ducts terminate
They open into the UG sinus on either side of teh sinus tubercle
What are the three primordia of the external genitalia
1. Genital tubercle=midline swelling of mesenchyme adjacent to phallic part of UG sinus
2. Urethral folds=paried swellings surronding the cloacal membrane
3. Labioscrotal swellings= swellings lateral to the urethral folds
Describe how testicular determination occurs
The SRY gene on the Y chromosome encodes testicular determining factor causing the supporting cells of teh genital ridge to differntiatie into sertoli cells which being to express Sox9 genes
Describe the formation of the seminiferous tubules
During week 7 sertioli and primordial germ cells organize into testicular cords. At or before puberty the cords develop a lumen and become the seminiferous tubles
Explain how the leydig cells are formed
At ~8 weeks, signals from the sertoli cells recruit adjacent mesenchyme cels to differentiate into the leydig cells
How is the tunica albuginea formed
The tunica albuginea is a condensation of mesenchyme cells that forms a thick connective tissue over the testis as it grows and rounds and separates from the mesonephros.
Describe the relocation of the testis
Occurs from the 7th week to the 9th month,
descend into the scrotum by shortening of the gubernaculum
(failure= cryptorchidism)
Describe the formation of the primordial follicle
Primordial germ cells enter the meiotic phase and become oocytes. The oocytes produce FIGa which stimulates the supporting cells to become follicle cells. The oocyte an dits surronding follicle cells form a primordial follicle
What is the role of the primordial follice in oocyte cell cycle?
The follicle secretes Meosis inhibiting factor that arrests the oocyte in prophase
What factors ensure that the interstital cells of a female become theca rather than leydig cells
Wnt 4
What male structures derived from the mesonephric ducts
duct of epididymis, ductus deferens, ejaculatory duct
What male structures develop from the mesonepheric tubles
efferent ductules
How does the prostate gland develop
Develops during week 10 as epithelial buds of endoderm form the prostatic urethra
How do the seminal vesicles form
Originate as outgrowth of the mesonephric duct before it enters the prostate gland.
What do the paramesonephric ducts become in males
mostly nothing, MIS causes them to atrophy
note that the cranial part of the prostatic utricle is derived from them
What do the mesonephric ducts become in females
nothing, absence of testosterone causes them to atrophy
What is the origin of the uterus
Lack of MIF allows development of the paramesonephric ducts. The inferior 1/3's fuse together forming the uterovaginal primordium which becomes the uterus
how is the vagina formed
Dual origin
1. Fused inferior 1/3 of paramesonephric ducts becomes uterovaginal primordium which forms the eipthelial lining of the upper vagina
2. The UV primordium contacts the pelvic part of the UG sinus forming the sinus tubercle. Sinovaginal bulbs extend dorsally form the tubercule and fuse forming the vaginal plate which becomes the epithelial lining of the lower vagina.
The surronding mesenchyme forms the rest of the tissue.
The fused paramesonephric ducts (uterovaginal primordium) become which part of the vagina
epithelial lining of the upper part
The sinovaginal bulbs derived from the sinus tubercle form which part of the vagina
epithelial lining of the lower part
What separates the lumen of the vagina from the UG sinus
hymen (note formation of a complete septum= imperforate hymen)
Vaginal atresia is most likely the result of problems with which structure
Failure of the paired paramesonehric ducts to form the unpaired uterovaginal primordium
In males, the pelvic part of the UG sinus becomes
1. distal part of prostatic urethra
2. membranous urethra
In females, the pelvic part of the urethra becomes
1. epithelial ling of the inferior part of the vagina
In males, the phallic part of the UG sinus becomes...
the epithelial lining of the penile urethra
In females, the phallic part of the UG sinus becomes
the vestibule of the vagina
What structures give rise to the phallus
Derived from the genital tubercle which is a midline swelling derived from the mesenchyme adjacent to the phallic part of the UG sinus
What two structures contribute to the formation of the penis
urethral plate and urethral folds
What is the precursor of the scrotum
labioscrotal swellings
What is hypospadias? What causes it?
The urethral opening is located on the ventral surface of the penis proximal to the tip. It occurs becaomes of incomplete fusion of the urethral folds.
What is epispadias?
In epispadias the urethra and penis are open dorsally.
What is the precursor of the clitoris
the phallus
What is the precursor of the epithelial linging of the vestibule of the vagina
the phallic portion of teh UG sinus
What do the urethral folds form in females
labia minora
What do the labioscrotal folds become in females
labia majora
What is the difference between a hermophrodite and a pseudohermaphrodite
herm=induvidual with charateristics of both sexes
pseudo= gonads and sex chromosomes don't match secondary characteristics
What are the characteristics of a true hermaphrodite
-both ovarian and testicular tissue (ofen as ovotestis)
-may have uterus on one side and vas deferens on the other
-external genitalia may be ambigous, but penis is often present
What might be a genetic explanation for a true hermaphordite
mosaicism where two distinct cell lines develop
or XX induvidal with remnant Y from abnormal crossover during clevage
What are the characteristics of a female pseudohermaphrodite
genetic female who has ovaries but masculine external genitalia
What might be a cause of female pseudohermaphrodites
-exposure to increased androgen
intrinsic= congenital adrenal hyperplasia
What are the charateristics of a male pseudohermaphrodite
genetic male who has testis but feminized or ambigous external genitalia
List some causes of male pseudohermaphrodites
1. insufficent androgen production due to enzyme defect
2. decrease in MIS
3. Androgen insensitivity syndrome
What are the characteristics of an induvidual with Kinefelter XXY
-seminiferous tubule dysgensis and gynecomastia due to androgen deficiency
tall, long limbs, mental "dullness"
What are the characteristics of pure gondal dysgenesis
Streak gonads and female genitalia
-in XY induviduals it is because of a mutation that inhibits SRY expression
-XO geneotype= Turner's syndrome
Describe mixed gondal dysgenesis
-ranges from turner like to male
-can have testis on one side and undifferentiated gonad on the other as well as a uterus
-may be varient of true hermaphrodism
What is the cause of XX,XY sex reversed
xx=> male, xy=> female
may be due to Z gene, an inhbitor for male pathway
mutation of lack of Z gene in an XX induvidual would result in male development
1. The hormone responsible for the external genitalia is:
a. Progesterone
b. Dehydroepiandosterone
c. Androstenedione
d. Testosterone
e. Dihydrotestosterone
e. Dihydrotesterone
2. Which one of the following is NOT a characteristic of complete androgen insensitivity in a 28 year old phenotypic female?

a. Increased LH
b. Normal adult female testosterone level
c. Complete breast development
d. Absent or sparse pubic hair
e. Undecended testes
b. Normal adult female testosterone level (testerone level will be like that of male)
This person is a genetic male but their cells are insensitive to androgne.
3. Deficiency of 17α-hydroxylase is:

a. autosomal dominant
b. X-linked dominant
c. X-linked recessive
d. autosomal recessive
e. a multifactorial disorder
d. autosomal recessive (Dr. Robb says all enzyme deficiencies are autosomal
4. Mullerian-inhibiting substance is secreted by:
a. Leydig cells
b. spermatogonia
c. sertoli cells
d. granulosa cells
e. endothelial cells
c. sertoli cells (of the testes)
What does aromatase do?
converts testoderone to estradiol
What is the "peritoneal" relatinoship of the gonads
retroperitoneal
what is the origin of the tunica albuginea
-forms under the coelomic epitheium as fibrous connective tissue by week 7
-derived from intermediate mesoderm
what is the genotype of an induvidual with mixed gonadal dysgenesis
45X/46XY
What might be the ause of a bicornuate uterus (partially unfused uterus)
A partial duplication of the uterus but the distal 1/3 of the paramesonephric ducts still fused.
The superior part of the body is duplicated while the inferior part is fused.
What happens if the wall between the two fused paramesopheric ducts fails to atrophy
the cavity of the uterus may be complelety or partially divided by a midline septum