Protein and AMino Acid Metabolism IV Flashcards Preview

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Flashcards in Protein and AMino Acid Metabolism IV Deck (34):
1

actual or function vit B12 defiency results in what

progressive neurlogical impariemnt

2

there is a strong correlation between high levels of homocyseine and the development of what disease - most likely as a marker

atheroslerosis

3

what coenzymes/vitamins does the homocysteine metabolism require

vit B6, B12, foalte

4

waht does valine catabolism producee

propionyl CoA

5

what does isoleucine catabolism produce

propionyl CoA adn acetyl CoA

6

what does catabolism of leucine prodcue

acetyl-CoA and acetoacetate

7

what does catabolism of BCAA outside of liver require

branched chain amino transferases, BCKDH

8

maple syrup urine disease is caused by waht

defect in BCKDH compelx

9

symptoms of maple syrup urine diseas

poor feeding, vomiting, slow/irregular breathing, ketoacidosis

10

how do you treat maple syrup urinse disease

reduced BCAAs in diet

11

what population is maple syrup urine disease more common in

old order mennonite

12

what casues tyrosinemia II

tyrosine amino acid transferase defect

13

what casues alcaptonuria

homogentisate oxidase defect

14

what casues tyrosinemia I

fumarylacetoacetate hydrolase defeect

15

symptoms of tyrosinemia II

keratitis
photophobia
painful skin lesion
intellectual disbiality

16

symptoms of alkaptonuria

black urine

17

symptoms of tyrosinemia I

liver, kidney, neurologic disfunction

18

srouces of ammonium ions

transfer of alpha mino groups to glutamate durign aminotransferase reaction
deamination of serine, cysteine, histidien and theronein
action of glutaminase and asparginase

19

what happens to most of the ammonium inos genreated during catabolism

incorporating into urea by liver

20

during normal metabolism the liver mitochondria genereate what

HCO3, CO2, NH4, ATP

21

what is the rate determing step of urea synthesis

formation of carbomoyl phosphate

22

what does carbamoyl phosphate synthetase I require

N acetylglutamate

23

how is the urea cycle linked to the TCA cycle

release of fumarte goes to TCA cycle which releases oxaloacetate which converts into aspartate which goes into urea cycle

24

when is the interstinal-renal axis established

postnatally

25

what is the only organ that can perform the entire urea cycle

liver

26

when is arginine essential in adults

conditions that impact function of small intestine or kidney

27

citrullinemia results from waht

defect in arginnosuccinate synthase

28

defect in what causes argininosuccinic aciduria

argininosuccinate lyase

29

what causes hyperargininemia

defect in arginase

30

how do you treat newborns with early cycle defects

protein restriction
hemodialysis
phenylbutyrate and benzoate

31

symptoms of hyeprammonenia

lethargy, stupar, vomiting, convulsions

32

what important thing is happening in astrocytes

main site of brain where glutamate is converted to glutamine

33

what happens after 10-12 horus of fasting

hepatic gluconeogensis required to supply glucose

34

the action of BCAT on BCAAs is a major source of what

nitrogen