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ENDO I > biosynthesis of steroid hormones > Flashcards

biosynthesis of steroid hormones Flashcards

1
Q

What does the adrenal medulla produce

A

catecholamines, norepi and mainly epi

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2
Q

what is synthesis of catecholamines regulated by

A

CRH-ACTH axis

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3
Q

ACTH stimulates what

A

synthesis of DOPA

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4
Q

High levels of cortisol have what affect on catecholamines

A

PMNT enzyme increases, which converts norepi to epi

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5
Q

Where is the synthesis of norepi

A

granules

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6
Q

where is the synthesis of epi

A

in the cytosol

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7
Q

What are the 2 enzymes in catecholamine degradation

A

MAO

COMT catecholamine O methyltransferase

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8
Q

When assaying for catecholamines what are you usually measuring

A

the degradation products in the urine

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9
Q

How are adrenergic R classified

A

potency
downstream signaling pathways
location and density

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10
Q

What is the role of activated alpha 1 R

A

vasc sm mm contraction

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11
Q

What is the role of activated alpha 2 R

A

inhibits norepi and insulin release

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12
Q

What is the role of activated B1 R

A

increase CO

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13
Q

What is the role of activated B3 R

A

increase glucose output and vasodilates

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14
Q

constant stimulation of alpha R have what effect on vasculature

A

chronic venoconstriction

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15
Q

what is the main role of cortisol

A

increase plasma glucose
responds to stress
influences glucose utilization, immune and inflammatory homeostasis

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16
Q

What hormones are produced in adrenal cortex

A

mineralocorticoids
glucocorticoids
Androgens (DHEA)

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17
Q

what is the backbone for all steroid hormones

A

cholesterol

18
Q

what is the first steroid produced from cholesterol

A

pregnalone

19
Q

What steroids can we get from pregnalone

A

progesterone via 3 beta hydroxylase

17 alpha hydroxylase can make it 17alpha pregnalone

20
Q

What converts progesterone to deoxy corticosterone

A

21 hydroxylase

21
Q

what is the purpose of 11 beta hydroxylase

A

take deoxycorticosterone to corticosterone

22
Q

what can corticosterone become

A

aldosterone (aldosterone synthase)
regulated by RAAS
cortisol by 17 alpha hydroxylase

23
Q

describe deficiency in 17 alpha hydroxylase

A

Everything is shunted to mineralocorticoid

cannot produce glucocorticoids or androgens

24
Q

describe deficiecy in 21 hydroxylase

A

cannot produce mineralocoritcoids and glucocorticoids

shunted into androgens

25
Q

describe deficiency in 11 beta hydroxylase

A

cannot produce glucocorticoids
some mineralocorticoids
shunted mainly to androgens

26
Q

What is congenital adrenal hyperplasia

A

group of autosomal recessive disorders

deficiency in either cotrisol, aldosterone or both

27
Q

When adrogen levels are high what happens

A

virilization

28
Q

what enzyme deficiencies can lead to high adrenal androgens

A

21-hydroxylase

11beta hydroxylase

29
Q

What enzyme deficiency leads to a high mineralocorticoid effect

A

17 alpha hydroxylase

hypertensive

30
Q

what enzyme deficiency leads to excess deoxycotricosterone

A

11 beta hydroxylase

31
Q

What are the signs of 21 hydroxylase deficiency

A 
salt wasting
hyponatremia
hyperkalemia
hypotension
CV collapse
virilization
32
Q

describe the two types of deficiencies of 21 hydroxylase

A

total or partial

33
Q

What does the 11 beta hydroxylase do

A

converts desoxycortisol to cortisol

34
Q

What happens in 11 beta hydroxylase deficiency

A

loss neg feedback inhibition and ACTH mediated adrenal androgen excess is the result

35
Q

What causes the hypertension in deficient 11 beta hydroxylase

A

increased secretion of deoxycorticosterone

36
Q

Is there any cortisol in a person with 11 beta hydroxylase deficiency

A

no

37
Q

how do you differentiate between 11 beta and 21 hydroxylase deficiencies

A

no salt wasting that you see in 21 hydroxylase

38
Q

what type of mutation is are the steroid hormone deficiencies

A

autosomal recessive

39
Q

What leads to hypertension and hypokalemia in 17alpha hydroxylase deficiency

A

excess intermediary steroids with mineralocorticoid activity

40
Q

what would be the aldosterone, K, Na, renin levels would be in someone with 21 hydroxylase deficiency

A

low aldosterone
low Na
high K
renin is increased

41
Q

17 y/o female, no menses, no pubic hair, no breast development, hypertensive
frequent headaches
hypokalemia, hypernatremia and metabolic alkalosis, low 17-ketosteroids, serum estrogen, increased progesterone, prenelone and 11 deoxycoricosterone and coricosterone

A

deficient 17 alpha hydroxylase, partial because later in life

ENDO I (9 decks)

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