Development of the Reproductive System Flashcards

1
Q

Until what week is the embryo indifferent (no sex)?

A

week 6

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2
Q

When does sexual differentiation begin?

A

week 7

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3
Q

When can female and male genitalia be recognized?

A

week 12

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4
Q

When is phenotypic differentiation complete in the embryo?

A

week 20

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5
Q

What are primordial germ cells?

A

progenitor cells of oogonia and permatogoonia

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6
Q

How do primordial germ cells migrate?

A

–>specified in epiblast, end up in yolk sac wall after gastrulation and body folding

–>migrate up dorsal mesentery to enter genital ridge of intermediate mesoderm

WEEK 5

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7
Q

Describe the location and appearance of primitive gonads during week 6

A

Initially a pair of longitudinal swelling in urogenital ridges of intermediate mesoderm
–>T10

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8
Q

How are primitive gonads formed?

A

proliferation and delamination of coelomic epithelium

condensation of underlying mesenchyme

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9
Q

What stimulates formation of somatic support cells?

A

primordial germ cells

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10
Q

How are somatic support cells formed during week 6?

A

proliferation and delamination of cells from coelomic epithelium of genital ridge

–> stimulated by primordial germ cells

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11
Q

What eventually differentiate into Sertoli cells in males?

A

somatic support cells

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12
Q

What eventually differentiates into follicle cells in females?

A

somatic support cells

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13
Q

What are the 2 sets of reproductive ducts that form within the intermediate mesoderm?

A

1: mesonephric tubules and duct (wolffian)
2: paramesonpehric duct (Mullerian)

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14
Q

What are the fxns of Wolffian/mesonephric ducts?

A

form within urogenital ridge

–>early collecting ducts for kidney

–>open into urogenital sinus

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15
Q

What are the fxns of Mullerian/paramesonephric ducts?

A

ducts form on both sides from invagination of urogenital ridge

–>cranially, open to coelomic cavity

–>caudally, meet with each other and attach to urogenital sinus

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16
Q

What is the key to sexual dimorphism?

A

Y chromosome

–>contains SRY that codes for testis

*also requires autosomes

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17
Q

What occurs in the absence of SRY gene?

A

formation of female reproductive organs

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18
Q

What does SRY turn on to drive male repro formation?

A

days 42-52

+ SOX9 expression in somatic support cells
—>Sertoli cell diff.

suppresses WNT4
–>pro-female gene

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19
Q

What make up testis cords?

A

-Sertoli cells
surround germ cells
-Primordial germ cells
-Differentiating myoepithelial cells

–>U shaped cords that develop into seminiferous tubules after birth

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20
Q

What are the rete testis?

A

like testis cords but don’t have germ cells

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21
Q

How are the testis cords linked to developing epididymis and vas deferens?

A

Testis cord attaches to rete testes–>connected to efferent ductules (remnant of mesonephric tubules)

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22
Q

What are derivatives of the mesonephric duct?

A

epididymis

vas deferens

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23
Q

Primordial germ cells differentiate into _______

A

type A spermatogonia 3 months after birth, hault until puberty

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24
Q

What makes fetal Leydig cells?

A

subset of intertubular cells differentiate into fetal Leydig cells
–>produce testosterone during week 9-10 to influence genital ducts and external genitalia

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25
Q

When do fetal Leydig cells start making testosterone?

A

weeks 9-10

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26
Q

What cells express anti-Mullerian hormone?

A

Sertoli cells

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27
Q

What is the fxn of anti-Mullerian hormone?

A

drives regression of paramesonephric ducts

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28
Q

When are paramesonephric ducts inhibited by anit-mullerian hormone?

A

weeks 8-10

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29
Q

What do remnants of paramesonephric duct make?

A
  • appendix testis

- prostatic utricle

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30
Q

What do mesonephric tubules and ducts form?

A
  • Efferent ductules from remaining mesonephric tubules
  • Epididymis
  • Vas deferens
  • Seminal vesicles
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31
Q

What drives mesonephric tubule remnants?

A

Leydig cell-derived testosterone

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32
Q

What is the paradidymis?

A

remnant of lower mesonephric tubules that are NOT used to make efferent ductules

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33
Q

What drives development of external genitalia?

A

DHT via 5a-reductase

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34
Q

What does DHT promote formation of?

A
  • penis
  • scrotum
  • prostate
  • ->testicular descent
35
Q

What drives fetal Leydig cells to produce testosterone?

A

HCG

36
Q

What are the fxns of ADULT Leydig cells?

A

generate androgens–> spermatogenesis

masculinization of brain and male sexual behavior

37
Q

What regulates testosterone production at puberty?

A

LH

38
Q

What germ layer makes up seminal vesicles?

A

intermediate mesoderm of urogenital ridge

39
Q

What is formed from buds emanating from the mesonephric duct?

A

seminal vesicles

40
Q

What forms from buds emanating from endoderm of urogenital sinus (within future region of pelvic urethra)?

A

prostate gland

–>week 10

41
Q

What germ layer makes up prostate gland and bulbourethral glands?

A

endoderm of urogenital sinus

42
Q

What forms from endoderm buds?

A

bulbourethral glands

43
Q

What is ectodermal-covered mesodermal swelling at distal end of phallic urogenital plate?

A

genital tubercle

–>growth depends on DHT

44
Q

How is the urogenital plate formed?

A

Cloacal membrane ruptures, roof of the phallic segment expands along lower surface of genital tubercle as it enlarges

–>endodermal extension under genital tubercle

45
Q

What drives lengthening and growth of genital tubercle and fusion of labioscrotal swellings?

A

DHT

46
Q

How is the tubular penile urethra formed?

A

urogenital folds fuse midline from perineal to gland penis

–>converts urethral groove into penile urethra

47
Q

Where is the urethral groove?

A

ventral surface of urogenital plate

48
Q

Where are the labioscrotal swellings in relation to urethral folds?

A

on either side

49
Q

What contains gonadal vessels, N, lymph to testis at T10?

A

suspensory ligament

50
Q

What connects developing gonad to base of labioscrotal swellings?

A

gubernaculum

–>eventually shortens to pull testes into scrotum

51
Q

Describe hypospadias

A

most common male birth defect after cryptorchidism

–>failure of urethral folds to close properly

*urethral opening on underside of penis instead of tip

52
Q

Describe epispadias

A

d/t improper ventral body closure

–>urethra opening on top of penis instead of tip

53
Q

In absence of SRY, somatic support cells express _________

A

WNT4 and FOXL2

54
Q

What is the fxn of FOXL2?

A

continually suppresses any SOX9 expression

–>maintains female gonad differentiation by continually suppressing Sertoli and Leydig formation

55
Q

What induces somatic support cells to become follicular cells?

A

developing oocytes

56
Q

What is responsible for arresting primary oocytes in prophase 1 of meiosis until puberty?

A

surrounding follicle cells

57
Q

Are paramesonpehric ducts retained in males or females?

A

females

58
Q

What are the remnants from mesonephric ducts in females?

A

epoophoron and paraoophoron near ovary

59
Q

Describe uterine tube formation

A

Paramesonephric ducts fused at caudal end by week 9-10

  • ->fusion of inferior end forms uterus
  • ->un-fused portion forms uterine tubes
60
Q

What are uterus anomalies d/t improper fusion of paramesonephric ducts?

A

1: Didelphys (double uterus)
2: Bicornate uterus (2 bodies, 1 cervix)
3: Double uterus and double vagina
4: Double uterus
5: Unicornate uterus (1 uterine tube)
6: Septated uterus

61
Q

What do the paramesonephric ducts form?

A

uterine tube
uterus
vagina

62
Q

What is included in external female genitalia?

A

labia
clitoris
lower vagina

63
Q

Describe formation of the vagina

A

Paramesonephric duct contacts urogenital sinus–> prolif and formation of sinuvaginal bulb

sinuvaginal plate lengthens, canalized

64
Q

What germ layer makes up the vagina (except upper fornix)?

A

endoderm

65
Q

Describe vaginal agenesis

A

Failure of normal sinuvaginal bulb development or canalization d/t agenesis of paramesonephric ducts

66
Q

What aspects of the phallic segment of urogenital sinus are the same in men and women initially?

A

urogenital plate
glans plate
genital tubercle

67
Q

Why is there no lengthening of the genital tubercle or fusion of labioscrotal/urogenital folds in females?

A

no DHT (via no testosterone)

68
Q

What forms the labia minora?

A

urogenital folds

69
Q

What forms the labia majora?

A

labioscrotal folds

70
Q

What separates urethra from vaginal opening?

A

differential growth and expansion of tissue

71
Q

What ligament is the portion of gubernaculum between the gonad and uterus?

A

round ligament of ovary

72
Q

What ligament is the portion of gubernaculum between the uterus and base of labioscrotal swellings?

A

Round ligament of uterus

73
Q

What restricts further ovarian descent?

A

gubernaculum attachment via round ligaments

74
Q

What is attached to the ovary during its descent?

A

suspensory ligament superiorly

gubernaculum connecting gonad to labioscrotal swelling (majora)

75
Q

What is a double fold of peritoneum (from urogenital ridge) that supports the uterus and ovary?

A

broad L
mesovarian
mesosalpinx

76
Q

Describe pathogenesis of 46 XY DSDs

A

testes but phenotypicallly female

  • -> inadequate testosterone synthesis (17B deficienty)
  • ->androgen insensitivity syndrome d/t loss of fxn of androgen receptors
  • ->5a-reductase deficienty (no DHT)
  • ->mutations in anti-mullerian hormone
77
Q

Describe mutations in AMH that cause DSDs

A

retention of paramesonephric ducts–>individuals have uterus and uterine tubes along with male ducts and external genitalia

78
Q

Describe 5a-reductase deficiency in DSDs

A

AR

  • normal testis and duct system
  • underdeveloped external genitalia
79
Q

Describe consequences of androgen insensitivity syndrome

A
  • have testes but no spermatogenesis
  • @ puberty, testosterone converted to estradiol to + female secondary characteristics
  • produce AMH so no uterus or tubes but vagina is short and ends blindly
  • testis found in inguinal or labial regions increases risk of cancer
80
Q

What is the most common cause of 46 XX DSDs

A

congenital adrenal hypoplasia

–>deficiency in 21-hydroxylase–>excess ACTH–>excess androgen production

81
Q

Describe sx of 46 XX DSDs

A

ovaries
female external genitalia masculinized
–>can look like hypospadias

82
Q

Describe ovotesticular disorders

A
  • have both testicular and ovarian tissue
  • ambiguous external genitalia or predominantly female
  • have uterus

**usually karyotype 46XX

83
Q

What are causes of ovotesticular disorders?

A

1: Translocation of piece of Y chromosome onto X chromosome
2: subset of cells may have mutation in Y chromosome
3: anomaly in sex determination and differentiation of primordial germ cells

84
Q

What is associated with extrophy of the bladder?

A

epispadias