BCHM 1 Nitrogen Metabolism Flashcards by Ross Larson

Q

Nitrogen balance

AA pool comes from?

first step of nitrogen removal?

A

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Q

Nitrogen balance

oxidative deamination via?

not to be confused with?

A

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Q

Nitrogen removal: urea cycle

carbon skeleton souce for urea cycle?

nitrogen source?

location of glutaminase?

where does fumarate go?

A

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

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Q

Nitrogen removal.

glomerular filtration process by which kidneys do what?

Diseases caused by problems in AA resorption

Double whammy effect of this?

A

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Q

where does reabsorption of AA and glucose happen?

what causes reuptake?

Hartnup and cystinuria defect?

both cause what?

A

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

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Q

Hartnup disease

Autosomal?

defect in?

major AA?

symptoms?

triggered by?

what precedes attack?

treatment?

A

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

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Q

tryptophan is derivative of?

A

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Q

Treatment of hartnups

A

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Q

Cystinuria

what causes it?

autosomal?

which AA?

results in?

positive test?

patient presents with?

what can help treat?

A

COAL

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Q

cystinuria

two variants

A

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Q

phenylalanine metabolism

what can errors cause?

what does it ultimately form?

A

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Q

PKU

caused by?

common?

what is the problem?

symptoms?

treatment?

secondary PKU?

A

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Q

treatment of PKU

needs to start when?

instituted if?

with milder forms of PKU?

A

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Q

Tetrahydrobiopterin/dihydrobiopterin

is a what?

essential in production of what?

does what in phenylalanine metabolism

defects lead to?

used in treatment?

A

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Q

Tyrosinemias

when is it?

transient tyrosinemias due to?

Type 1 from? symptoms?

type II from? symptoms?

type III from? symptoms?

most common?

treatment?

A

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Q

Alkaptonuria

caused by?

autosomal?

causes what?

triad?

A

Q

ammonia toxicity

from?

causes?

A

Q

Gout

characterized as?

primary?

secondary?

results in?

treatments?

A

Q

Hyperammonemia

defects in?

treatment?

A

Q

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?

A

Q

carbamoyl phosphate synthetase I and II

A

Q

direct and indirect bilirubin

what is direct and indirect bilirubin?

how do we test for it?

difference between two tells us?

A

Q

heme break down

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

transported to? by?

released as?

excreted as? (urine/feces)

A

Q

Jaundice of newborns

what happens?

severe if?

treatment?

A