Development of the Reproductive System (Brauer) Flashcards

1
Q

What are primordial germ cells

A

Cells of the epiblast that are initially localized w/in the walls of the yolk sac but migrate up the dorsal mesentery into the genital ridge of the intermediate mesoderm. They develop into oogonia and spermatogonia.

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2
Q

How are somatic support cells formed

A

Primordial germ cells stimulate cells from the coelomic epithelium of the genital ridge to proliferate and delaminate into Somatic support cells

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3
Q

What do somatic support cells develop into in males & females

A

Males - Sertoli cells

Females - Follicle cells

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4
Q

What are the two sets of reproductive ducts that form within the intermediate mesoderm

A

Mesonephric ducts (Wolffian) & Paramesonephric ducts (Mullerian)

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5
Q

What structures are derived from the mesonephric duct

A

Epididymis

Vas Deferens

Seminal Vesicle

Ejaculatory duct

Mesonephric duct is lost in females so only male structures formed

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6
Q

What structures are derived from the Parameonephric duct

A

Oviduct

Uterus

Upper Vagina

Parameonephric duct is lost in males so only female structures formed

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7
Q

What is the role of SRY in sex determination

A

It is the testis-determining factor gene which is located on the Y chromosome. In it’s absence, development is female.

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8
Q

What is the effects of SRY in males

A
  1. Turns on SOX9 expression in somatic support cells which triggers them to differentiate into Sertoli cells
  2. Suppresses Wnt4 (pro-female gene)
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9
Q

What is the effects of the abscent of SRY in females

A

Since the SRY gene is abscent, somatic support cells express Wnt4 and FOXL2 (supresses SOX-9)

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10
Q

What do a subset of intertubular cells differentiate into in males

A

Leydig cells (recruited by Sertoli cells)

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11
Q

What is the role of Sertoli cells in male development

A

They express Anti-Mullerian hormone (AMH) which causes the paramesonephric duct to degenerate

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12
Q

What are the remnants of the paramesonephric duct

A

Appendix testis & proststic utricle

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13
Q

What do Fetal Leydig cells do

A

They produce testosterone which triggers the mesonphric tubules and ducts to form:

  • Effernet ductules
  • Epididymis
  • Vas deferens
  • Seminal vesicles
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14
Q

How are external male genitalia developed

(penis, scrotum, prostate & testicular descent)

A

Testosterone is converted to dihyrotestosterone (DHT) by 5-alpha-reductase

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15
Q

What is the role of adult Leydig cells

A

Produce androgens that initiate and maintain:

Spermatogenesis

Masculinization of the brain

Male sexual behavior

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16
Q

What germ layers are semial vesicles formed from

A

Intermediate mesoderm of urogenital ridge

Seminal vesicles are buds coming of the mesonephric duct hence deried from intermediate mesoderm

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17
Q

What germ layers are prostate glands from

A

Endoderm buds of urogenital sinus

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18
Q

What is the urogenital plate

A

The roof of the phallic portion of the urogenital sinus that is lined by endoderm. It expands along the lower surface of the genital tubercle as the genital tubercle enlarges

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19
Q

What germ layer are the bulourethral glands formed from

A

Endodermal buds

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20
Q

How is the genital tubercle form?

A

Ectodermal-covered mesoderm swelling at ventral and cranial end of phallic portion of urogenital plate.

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21
Q

What is the glans plate?

A

Remnants of cloacal membrane at the ventral end of urogenital place and adjacent genital tubercle

22
Q

What are the pair of swellings that develop on either side of the urogenital plate through aan expansion of mesoderm underlying the ectoderm

A

Urogenital folds

23
Q

Fusing of the urogenital folds with each other forms what structure

A

Urethra

24
Q

What promotes lengthening and growth of the genital tubercle to form the penis & fusion of labioscrotal swellings to form the scrotum

A

dihydrotestosterone (DHT)

25
Q

What is hypospadias

A

A common male birth defect that is a result of the failure of the urogenital (urethral) folds to close properly

26
Q

What is Epispadias

A

Opening of the urethra on the superior aspect of the penis. Cause is unknown but may be due to improper ventral body closure or formation of too large of a cloacal membrane to be covered when it ruptures

27
Q

In females, what gene is expressed and promotes the female reproductive system?

A

WNT-4 & FOXL2

28
Q

What suppresses SOX-9 in females?

A

FOXL2

29
Q

What is the effect of the suppression of SOX-9

A

No SOX-9 = No Sertoli cells = No AMH = paramesonephric ducts retained

30
Q

What blocks oocytes in meiosis-1?

A

Follicle cells

31
Q

Absence of Leydig cells in females means what?

A

Mesonephric duct system is lost

32
Q

Fusion of the inferior paramesonephric ducts form what

A

Uterus

33
Q

The unfused superior portion of the paramesonephric ducts form what

A

Uterine tubes

34
Q

What is didelphys

A

Double uterus

35
Q

How is the vagina formed

A

Contact of paramesonephric duct to the urogenital sinus stimulates proliferation and formation of sinuvaginal bulb which is eventually canalized

36
Q

What germ layer is the vagina derived from

A

Whole thing is endodermally derived with the exception of the upper vaginal fornix which is mesodermally derived.

37
Q

What is vagina agensesis

A

Lack of a vagina due to failure of normal sinuvaginal bulb development and/or canalization

38
Q

What do the urogenital folds and labiosacral folds develop into in females

A

Urogenital folds - labia minora

Labioscrotal folds - labia majora

39
Q

What does the phallic segment of the urogenital sinus contain in females

A

Urogenital plate

Glans plate

Genital tubercle

Same as in males

40
Q

What is the broad ligament?

A

Peritoneal fold that attaches the uterus, fallopian tubes, and ovaries to the pelvis

41
Q

How does the broad ligament form?

A

Remaining tissue from the uterus and oviduct forms a double fold of peritoneum

42
Q

What does the round ligament of ovary connect?

A

Ovary to uterus

43
Q

What is the embryological origin of the round ligament of ovary?

A

Superior gubernaculum

44
Q

What does the round ligament of uterus connect?

A

Uterus to labia majora

45
Q

What is the embryological origin of the round ligament of uterus?

A

Inferior gubernaculum

46
Q

What is the term for the congenital condition in which development of chromosomal, gonadal and anatomical sex is atypical

A

Disorders of sex development (DSDs)

47
Q

What is it called when an individual has testes but their phenotype appears female

A

46, XY DSDs

48
Q

What are some causes of 46, XY DSDs

A
  1. Inadequate testosterone synthesis
  2. Androgen insensitivity syndrome
  3. 5 alpha-reductase deficiency
  4. Mutation in AMH or AMH receptor
49
Q

What are the characteristics of androgen insensitvity syndrome

A
  1. Common cause is loss of functional androgen receptors (X linked recessive)
  2. They have testes but no spermatogensis. Testosterone levels may be high due to a lack of negative feedback.
  3. At puberty, the testosterone is metabolized to estradiol initiating female secondary sexual characteristics
  4. Produce AMH so paramesonephric system is supressed = no uterus or uterine tube and the vagina is short and ends blindly.
  5. Testes usually found in inguinal or labial regions
  6. Increased risk of tumor formation in gonads
50
Q

What are the characteristics of 5- alpha-reducatse deficiency

A
  • Autosomal recessive
  • Have a 46 XY genotype
  • Normal testis and duct system since there is AMH and testosterone
  • Underdeveloped male external genitalia due to deficieny in DHT
51
Q

What are the characteristics of 46 XX DSDs

A
  • Presence of ovaries
  • Fetus produces excess androgens
  • Maculinzation of the female external genitalia; may even exhibit fusion of labia giving appearance of a scrotum.
  • Most common cause is congenital adrenal hyperplasia
52
Q

How does Congenital adrenal hyperplasia cause of 46 XX DSDs?

A

Deficiency in 21-hydroxylase leads to excess ACTH production (lack of negative feedback). Excess ACTH causes an excessive production of androgens.