Development of the Genital System _ PV Flashcards

1
Q

Timeline of Genital System

  • Week 1
  • Week 7
  • Week 12
  • Week 20
A

Week 1: indifferent embryo

Week 7: sexual differentiation begins

Week 12: Female and male genitalia can be recognized

week 20: phenotypic differentiation complete

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2
Q

What happens in Genital system at week 5?

A

Indifferent Gonad

primordial germ cells specified within epiblast

  • end up in yolk sac wall after gastrulation and body folding

migrate up dorsal mesentery to enter genital

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3
Q

What happens to genital system at week 6?

A

indifferent gonad

somatic support cells
Male: sertoli cells
Female: follicle cells

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4
Q

Where do the genital ducts form?

A

intermediate mesoderm of the urogenital ridge

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5
Q

Mesonephric duct

  • aka
  • what it makes
A

wolffian duct

  • part of early kidney development
  • epididymis, vas deferens, seminal vesicle, ejaculatory duct
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6
Q

Paramesonephric duct

  • aka
  • what it make
A

Mullerian Duct

  • uterine tube, uterus, upper vagina
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7
Q

What is the gene that determines sex?

- what happens when you do not have it>

A

SRY gene on Y chromosome

w/o SRY- development is female

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8
Q

Describe SRY

  • aka
  • how long is it active
  • where is it expressed
A

transcription factor

Testis determining factor

active from 41-52 days

expressed in somatic support cells (pre-sertoli)

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9
Q

Pathway of SRY

- paramesenphric duct

A

Sertoli cells-> SRY-> SOX9-> AMH and ABF-> regression of Mullerian duct bte week 8-10-> remnants are appendix testis and prostatic utricle

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10
Q

Pathway of SRY

- mesonephric duct

A

Leydig cells-> SRY-> testosterone-> Wolffian duct-> ductus deferens, epipdidymis, vas deferens, seminal vesicles

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11
Q

What are the two things Sertoli cells secrete?

A
  1. AMH ( regression of paramesonephric ducts)

2. Androgen binding factor ( spermatogonia-> spermatozoa)

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12
Q

Leydigs Cells
1. Fetal
FATESS
2.adult

A
  1. Fetal
    a. testosterone
    - week 8-12 driven by HCG of placenta
    - mesonephric duct-> ductus deferens, epididymis, seminal vesicle
    b. 5 a reductase
    - testosterone-> DHT
    - Genital tubercle-> penis
    - genital swelling-> scrotum
    - urethral epithelium-> prostate
  2. Adult Leydig cells
    - androgens-> initiation of spermatogenesis, masculinzation of brain, male sexual behavior
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13
Q

Parts of External Genetalia

A
  1. Urogenital plate
  2. Glans Plate
  3. Genital Tubercle
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14
Q

Urogenital Plate

A

rupture of cloacal membrane opens phallic portion of urogenital sinus to exterior; forming endodermally-lined plate

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15
Q

Glans plate

A

remnants of cloacal membrane at the ventral end of urogenital plate and adjacent genital tubercle

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16
Q

Genital tubercle

A

ectodermal-covered mesodermal swelling at ventral and cranial end of phallic portion of urogenital plate

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17
Q

Role of DHT

A

lengthen genital tubercle and fusion of labioscrotal swellings to form scrotum

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18
Q

Testis descent

  • when
  • 2 component
A

week 10

supspensory= gonada; vessels, nerves, lymphatics

gubernaculum- shortens and becomes anchoring ligament of testis of scrotum

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19
Q

Hypospadias

A

most common birth defect after cyrptochidism

  • failure of neural tube folding
  • get penile shaft
20
Q

Episadias

A

rare

  • extrophy of bladder
  • ventral body fold defect
21
Q

Ovary development

  • genes
  • cell maturation pathway
A

WNT4-> FOXL2 suppresses SOX 9

primordiall germ cells -> oogonia-> oocytes-> follicle cells surround oocytes

no sertoli or leydig cells

  • paramesonephric persist
  • mesonephric duct system lost
22
Q

FOXL2 pathway

A

FOXL2-> inhibit SRY to AMH-> persistence of Mullerian (paramesonephric) duct-> fallopian tubes, uterus, vagina ( upper portion)

23
Q

Uterus formation

  • when
  • describe
A

week 9-10

  • endoderm form sinovaginal bulb
  • fusion of inferior paramesonephric ducts form the uterus
  • uterine tubes are the unfused superior portion of the paramesonephric ducts
24
Q

Uterus anomalies

A

didelphys- term for double uterus

25
Q

Embryological layer of upper vagina, lower vagina

A

upper vagina= mesoderm

lower vagina= endoderm

vaginal plate eventually canalized

26
Q

Vaginal agenesis

A

failure of normal sinovaginal bulb to development or failed canalization

27
Q

Female external genitalia development

A

no testosterone= no DHT=> no lengthening of genital tubercle and no fusion of urogenital folds or labioscrotal swellings

28
Q

Formation of Broad Ligament

A
  • midline fusion of paramesonephric ducts brings lower urogenital ridge within pelvic cavity and it is covered with peritoneum
  • upon completion of uterus and oviduct formation, the remaining tissue thins forming a double fold of peritoneum ( broad ligament) support uterus and ovary
29
Q

Round Ligament of Ovary and Uterus

A

Superior gubernaculum= round ligament of ovary ( connects ovary to uterus)

Inferior gubernaculum= round ligament of uterus ( connects uterus to labia majora)

30
Q

Indifferent gonad

  • male
  • female
A

Male: Testis

Female: ovary

31
Q

Primordial germ cells

  • male
  • female
A

male: spermatogonia
female: oocytes

32
Q

Somatic support cells

  • male
  • female
A

male: sertoli cells
female: follicular cells

33
Q

Stromal cells

  • male
  • female
A

male: leydig cells
female: thecal cells

34
Q

Gubernaculum

  • male
  • female
A

Male: gubernaculum testis

Female:

  • round ligament of ovary
  • round ligament of uterus
35
Q

Mesonephric tubules

  • male
  • female
A

male: efferent ducts
female: epoophoron, paroophoron

36
Q

Mesonephric duct

  • male
  • female
A

Male:

  • epididymis
  • vas deferens
  • seminal vesicle
  • ejaculatory duct

female:
gartner duct

37
Q

Paramesonephric duct

  • male
  • female
A

Male:

  • testis
  • prostatic utricle

Female

  • fallopian tube
  • uterus
  • vagina
38
Q

Urogenital Sinus

  • Male
  • Female
A

Male

  • prostatic urethra
  • prostatic gland
  • BU gland

Female:

  • membranous urethra
  • paraurethral gland
  • greater vestibular gland
39
Q

Genital tubercle

  • male
  • female
A

male:

  • glans penis
  • corpus cavernousa
  • corpus spongiosum

Female:

  • glans clitoris
  • corpus cavernousa of clit
  • bulbospongiosum
40
Q

Urogenital folds and glans plate

  • male
  • female
A

male:
penile urethra

female:
labia minora

41
Q

labioscrotal fold

  • male
  • female
A

male
- scrotum

female
- labia majora

42
Q

Pseudohermaphroditism

A

intersex

genotypic sex masked by phenotypic appearance resembling the opposite sec or having opposite reproductive organs

43
Q

46 XY DSD ( male)

A

testis but phenotype female

  • inadequate testosterone synthesis ( 17 B hydrosteroid DH)
  • androgen insensitivity syndrome
  • 5a reductase deficiency
  • mutation in AMH or AMH receptor
44
Q

Androgen insensitivity Syndrome

A

x linked recessive

-testis but no spermatogenesis= testosterone levels may be high

testerone is metabolized to estradiol=> female secondary characteris but amenorrhea

  • produce AMH so paramesonephric system is suppressed
45
Q

5 a reductase deficiency

A

46 XY

autosome recessive

normal testis and duct system

underdeveloped male external genitalia
- short penis

no effect on mesonephric system and gonadal development

46
Q

Female DSD

A

46 XX genotypes

have ovaries

excessive androgens=> fusion of labia, giving appearance of scrotum

Congenital adrenal hyperplasia
- 21 hydroxylase deficiency

47
Q

Ovotesticular Disorders

A

true intersexuality

both testicular and ovarian tissue

ambiguos external genital and predominantly female

causes:

  • translocation of piece of Y onto X
  • subset may have mutation in Y
  • anomaly in sex determination and differentiation of primordial germ cells