amino acid trasnport

Question 1

how do amino acids get into the gi

Answer 1

Na coupled secondary transort

Question 2

what does the amino acid group join with to produce 2-ketoacid?

Answer 2

PLP

Question 3

what types of amino acids are created when 2-ketoacid joins again with PLP

Answer 3

a-ketoglutarate that turns into

glutamic acid

Question 4

glutamate produces the first nitrogen that goes into Urea synthesis where and in what form? what enzyme and cofactors are important?

Answer 4

in the liver as NH4+

glutamate dehydrogenase with cofactors NAD and NADH

Question 5

what are the important carriers of nitrogen in the blood?

Answer 5

aspartate
glutamine
glutamate

Question 6

tissue location of arginase? important for?

Answer 6

Arginine synthesized in the liver typically used for urea synthesis, it can also be produced in the kidney and interstines and other tissues but because other tissues DON’T have arginase, urea doesn’t end up synthesized there rather only in the liver. Arginine can also be decarboxylated to form agmatine (intermediate for some polyamines and also appears to neurotransmitter activity in the brain, may have clinical siginfican used in pain mgmt/adiction)

Question 7

where is urea produced?

Answer 7

liver

Question 8

negative nitrogen balance occurs

Answer 8

in starvation

Question 9

positive nitrogen balance occurs

Answer 9

rapid growth or after trauma for reconstruction

Question 10

making urea in the liver starts with combining bicarbonate with ammonia and requires 2 ATPs to make____ and requires what enzyme and cofactor?

Answer 10

makes Carbamoyl phosphate

requiring Carbamoyl phosphate synthetase I and N-acetyleglutamate

Question 11

what are the short term regulators of urea cycle?

Answer 11

activating N-acetyl glutamate (cofactor for carbamoyl phosphate synthetase I in urea creation) via carbamoyl phosphate

increased arginine concentrations allosterically acticate enzyme that makes the N-Acetyle Glutamate

Question 12

type I hyperammonemia is a deficiency in

Answer 12

Carbamoylphosphate synthetase I

tx: at birth baby needs arginine to activate N-acetylglutamate

Question 13

N-acetylglutamate synthetase deficiency leads to hyperammonemia because ____

Answer 13

Ammonia cannot be converted to carbamoyl phosphate then added with ornithine to make citrulline and then eventually make urea to be excreted

Question 14

The most commonly occuring Urea Cycle disorder is ______

Answer 14

Type 2 Hyperammonemia caused by Ornithine transcarbamoylase deficiency

Question 15

Type 2 Hyperammonemia caused by Ornithine transcarbamoylase deficiency is _______ linked recessive. causes increased ___acid.

Answer 15

Xlinked recessive

Orotic acid buildup from feeding into pyrimidine synthesis

Question 16

Classic Citrullinemia is a deficiency in

Answer 16

argininosuccinate synthetase

Question 17

classic citrillunemia presents with

Answer 17

epidoside hyperannomemia

vomiting
lethargy
ataxia
seizures
eventual coma

Question 18

what can help bind to NH4+ and tx hyperammonemia?

sodium____ or sodium______

Answer 18

Sodium benzoate

sodium phenylacetate

Question 19

Argininosuccinate lyase deficiency causes Argininosuccinic aciduria which presents with increased plasma and CSF of______ and you treat with _____

Answer 19

argininosuccinate

arginine and sodium benzoate

Question 20

Arginase deficiency can lead to hyperargininemia which is rare and can causes severe conditions as

Answer 20

progressive spastic quadriplegia

mental retardation