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Flashcards in BCHM 1 Nitrogen Metabolism Deck (24)
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1

Nitrogen balance

AA pool comes from?

first step of nitrogen removal?

2

Nitrogen balance 

oxidative deamination via?

not to be confused with?

3

Nitrogen removal: urea cycle

carbon skeleton souce for urea cycle?

nitrogen source?

location of glutaminase?

where does fumarate go?

 

we start with the deamination reaction from glutamate or glutamine to get NH3 which will become NH4 this reacts with bicarbonate to make Carbamoyl phosphate.

Fumarate is a TCA intermediate that is recycled back to aspartate 

4

Nitrogen removal.

glomerular filtration process by which kidneys do what?

Diseases caused by problems in AA resorption

Double whammy effect of this?

5

where does reabsorption of AA and glucose happen? 

what causes reuptake?

Hartnup and cystinuria defect?

both cause what?

  • re-absorption of AA and glucose occurs in PCT
  • filtrate through PCT transport proteins from the SLC (solute carrier) gene superfamily mediate reuptake of AA and glucose

6

Hartnup disease

Autosomal?

defect in?

major AA?

symptoms?

triggered by?

what precedes attack?

treatment?

The defective neutral AA transporter B AT1 encoded by the SLC6A19 gene on chromosome 5p15 requires either collectrin or angiotensin-converting enzyme 2 for surface expression in the kidney and intestine respectively

7

tryptophan is derivative of?

8

Treatment of hartnups

9

Cystinuria

what causes it?

autosomal?

which AA? 

results in?

positive test?

patient presents with?

what can help treat?

COAL

10

cystinuria 

two variants

11

phenylalanine metabolism

what can errors cause?

what does it ultimately form?

12

PKU

caused by?

common?

what is the problem?

symptoms?

treatment?

secondary PKU?

13

treatment of PKU

needs to start when?

instituted if?

with milder forms of PKU?

14

Tetrahydrobiopterin/dihydrobiopterin 

is a what?

essential in production of what?

does what in phenylalanine metabolism

defects lead to?

used in treatment?

15

Tyrosinemias 

when is it?

transient tyrosinemias due to?

Type 1 from? symptoms?

type II from? symptoms?

type III from? symptoms?

most common?

treatment?

16

Alkaptonuria

caused by?

autosomal?

causes what?

triad?

17

ammonia toxicity

from?

causes?

18

Gout

characterized as?

primary?

secondary?

results in?

treatments?

19

Hyperammonemia

defects in?

treatment?

20

Carbamoyl phosphate synthetase II

what is it?

involved in?

doesnt require what compared to other one?

defect in UMP synthase lead to?

this is different from before why?

21

carbamoyl phosphate synthetase I and II

22

direct and indirect bilirubin

what is direct and indirect bilirubin?

how do we test for it?

difference between two tells us?

23

heme break down

what is heme broken down to in spleen/ liver macrophage?

transported to? by?

released as?

excreted as? (urine/feces)

24

Jaundice of newborns

what happens?

severe if?

treatment?