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
Embryological layer of upper vagina, lower vagina
upper vagina= mesoderm lower vagina= endoderm vaginal plate eventually canalized
26
Vaginal agenesis
failure of normal sinovaginal bulb to development or failed canalization
27
Female external genitalia development
no testosterone= no DHT=> no lengthening of genital tubercle and no fusion of urogenital folds or labioscrotal swellings
28
Formation of Broad Ligament
- 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
Round Ligament of Ovary and Uterus
Superior gubernaculum= round ligament of ovary ( connects ovary to uterus) Inferior gubernaculum= round ligament of uterus ( connects uterus to labia majora)
30
Indifferent gonad - male - female
Male: Testis Female: ovary
31
Primordial germ cells - male - female
male: spermatogonia female: oocytes
32
Somatic support cells - male - female
male: sertoli cells female: follicular cells
33
Stromal cells - male - female
male: leydig cells female: thecal cells
34
Gubernaculum - male - female
Male: gubernaculum testis Female: - round ligament of ovary - round ligament of uterus
35
Mesonephric tubules - male - female
male: efferent ducts female: epoophoron, paroophoron
36
Mesonephric duct - male - female
Male: - epididymis - vas deferens - seminal vesicle - ejaculatory duct female: gartner duct
37
Paramesonephric duct - male - female
Male: - testis - prostatic utricle Female - fallopian tube - uterus - vagina
38
Urogenital Sinus - Male - Female
Male - prostatic urethra - prostatic gland - BU gland Female: - membranous urethra - paraurethral gland - greater vestibular gland
39
Genital tubercle - male - female
male: - glans penis - corpus cavernousa - corpus spongiosum Female: - glans clitoris - corpus cavernousa of clit - bulbospongiosum
40
Urogenital folds and glans plate - male - female
male: penile urethra female: labia minora
41
labioscrotal fold - male - female
male - scrotum female - labia majora
42
Pseudohermaphroditism
intersex genotypic sex masked by phenotypic appearance resembling the opposite sec or having opposite reproductive organs
43
46 XY DSD ( male)
testis but phenotype female - inadequate testosterone synthesis ( 17 B hydrosteroid DH) - androgen insensitivity syndrome - 5a reductase deficiency - mutation in AMH or AMH receptor
44
Androgen insensitivity Syndrome
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
5 a reductase deficiency
46 XY autosome recessive normal testis and duct system underdeveloped male external genitalia - short penis no effect on mesonephric system and gonadal development
46
Female DSD
46 XX genotypes have ovaries excessive androgens=> fusion of labia, giving appearance of scrotum Congenital adrenal hyperplasia - 21 hydroxylase deficiency
47
Ovotesticular Disorders
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