Protein and AMino Acid Metabolism IV

Question 1

actual or function vit B12 defiency results in what

Answer 1

progressive neurlogical impariemnt

Question 2

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

Answer 2

atheroslerosis

Question 3

what coenzymes/vitamins does the homocysteine metabolism require

Answer 3

vit B6, B12, foalte

Question 4

waht does valine catabolism producee

Answer 4

propionyl CoA

Question 5

what does isoleucine catabolism produce

Answer 5

propionyl CoA adn acetyl CoA

Question 6

what does catabolism of leucine prodcue

Answer 6

acetyl-CoA and acetoacetate

Question 7

what does catabolism of BCAA outside of liver require

Answer 7

branched chain amino transferases, BCKDH

Question 8

maple syrup urine disease is caused by waht

Answer 8

defect in BCKDH compelx

Question 9

symptoms of maple syrup urine diseas

Answer 9

poor feeding, vomiting, slow/irregular breathing, ketoacidosis

Question 10

how do you treat maple syrup urinse disease

Answer 10

reduced BCAAs in diet

Question 11

what population is maple syrup urine disease more common in

Answer 11

old order mennonite

Question 12

what casues tyrosinemia II

Answer 12

tyrosine amino acid transferase defect

Question 13

what casues alcaptonuria

Answer 13

homogentisate oxidase defect

Question 14

what casues tyrosinemia I

Answer 14

fumarylacetoacetate hydrolase defeect

Question 15

symptoms of tyrosinemia II

Answer 15

keratitis
photophobia
painful skin lesion
intellectual disbiality

Question 16

symptoms of alkaptonuria

Answer 16

black urine

Question 17

symptoms of tyrosinemia I

Answer 17

liver, kidney, neurologic disfunction

Question 18

srouces of ammonium ions

Answer 18

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

Question 19

what happens to most of the ammonium inos genreated during catabolism

Answer 19

incorporating into urea by liver

Question 20

during normal metabolism the liver mitochondria genereate what

Answer 20

HCO3, CO2, NH4, ATP

Question 21

what is the rate determing step of urea synthesis

Answer 21

formation of carbomoyl phosphate

Question 22

what does carbamoyl phosphate synthetase I require

Answer 22

N acetylglutamate

Question 23

how is the urea cycle linked to the TCA cycle

Answer 23

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

Question 24

when is the interstinal-renal axis established

Answer 24

postnatally

Question 25

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

Answer 25

liver

Question 26

when is arginine essential in adults

Answer 26

conditions that impact function of small intestine or kidney

Question 27

citrullinemia results from waht

Answer 27

defect in arginnosuccinate synthase

Question 28

defect in what causes argininosuccinic aciduria

Answer 28

argininosuccinate lyase

Question 29

what causes hyperargininemia

Answer 29

defect in arginase

Question 30

how do you treat newborns with early cycle defects

Answer 30

protein restriction
hemodialysis
phenylbutyrate and benzoate

Question 31

symptoms of hyeprammonenia

Answer 31

lethargy, stupar, vomiting, convulsions

Question 32

what important thing is happening in astrocytes

Answer 32

main site of brain where glutamate is converted to glutamine

Question 33

what happens after 10-12 horus of fasting

Answer 33

hepatic gluconeogensis required to supply glucose

Question 34

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

Answer 34

nitrogen