PROTEIN METABOLISM II

Question 1

metabolic fates of keto acids

S,G,K,M

Answer 1

synthetic pathway
glucogenic pathway
ketogenic pathway
miscellenaous pathway

Question 2

aplha keto acid + ammonia —> amino acid

keto acids with ammonia are going to form back your amino acids

Answer 2

synthetic pathway

Question 3

amino acids can be converted to glucose
keto acids form keto acid will enter krebs cycle the gluconeogenesis

Answer 3

glucogenic pathway

Question 4

amino acids can enter the ketogenic pathway which becomes ketone bodies

Answer 4

ketogenic pathway

Question 5

purely ketogenic amino acid

Answer 5

Leucine

Question 6

both glycogenic and ketogenic amino acids

Answer 6

Phenylalanine
isoleucine
tyrosine

tryptophan
lysine

Question 7

5 amino acids that could be converted to pyruvate

A
C
G
S
T

Answer 7

alanine
cysteine
glycine
serine
threonine

Question 8

5 amino acids that could be converted to alpha ketoglutarate

AGHP

Answer 8

arginine glutamine histidine proline

Question 9

phenylalanine and tyrsone are always together because you can easily convert phenylalanine to tyrosine through _________

Answer 9

oxygenation

Question 10

absence of the major enzyme ________ are of the causes of PKU

Answer 10

phenylalanine hydroxylase

Question 11

in alkaptonuria ________ cannot be converted to maleyacetoacetate

Answer 11

homogentisic acid

Question 12

triad of alkaptonuria

O,A,B

Answer 12

ochronosis
arthritis
black urine

Question 13

in albinism there is absence of the enzyme _______

Answer 13

tyrosinase

Question 14

Type 1 hepatorenal tyrosinemia is ______ deficiency

accumulation of maleyacetoacetate and fumarylacetoacetate

Answer 14

fumarylacetoacetate hydrolase deficiency

Question 15

Type II oculocutaenous tyrosinemia has __________ deficiency

Answer 15

tyrosine aminotransferase

Question 16

most important derivatives of tryptophan metabolism is _______

Answer 16

niacin

Question 17

due to b2 and b6 deficiency
which means Rough skin

Answer 17

pellagra

Question 18

give the patient tryptophan the measure amount of xanthurenic acid in the urine

increased xanthurenic acid indication of vitamin b6 deficiency

Answer 18

tryptophan load test

Question 19

substance than can be derived from tryptophan

it is a neurotransmitter, vasoconstrictor, and stimulates smooth muscle contraction

Answer 19

serotonin

Question 20

elevated in carcinoid syndrom (argentaffinoma)

Answer 20

5-hydroxyindolacetate

Question 21

derived from tryptophan
sleep molecule

Answer 21

melatonin

Question 22

impaired intestinal and renal absorption of tryptophan

tryptophan will go to large intestines instead of the small intestines which then will be converted to indole and skatole

Answer 22

blue diaper syndrome

Question 23

histidine can be converted to _________

Answer 23

glutamic acid

Question 24

in histidinemia there is _________- deficiency

Answer 24

histidase deficiency

Question 25

maple syrup branched chain ketonuria there is _______ deficiency odor resembles maple syrup

Answer 25

alpha ketoacid decarboxylase

Question 26

in isovaleric acidemia there is deficiency of _________ manifested by cheesy odor of the breathe and bodily fluids

Answer 26

isovaleryl CoA DH defieciency

Question 27

in hyperlisinemia (accumulation of lysine) thereis is ________ deficiency

Answer 27

saccharopine dehydrogenase deficiency

Question 28

in homocystinuria there is deficieny of ___________ while in cystathioninuria there is deficiency of __________

Answer 28

crystathionine synthetase crystathionase

Question 29

considered to be due to a renal transport defect affecting renal absorptive mechanisms for 4 amino acids - cysteine, lysine, arginine, and ornithine

Answer 29

cystinuria

Question 30

primary defect: impaired lysosomal function deposition of cystine crystals in many tissues and organs particularly the reticuloendothelial system

Answer 30

cystinosis

Question 31

glutamic acid can also come from the catabolism of histidine via ______ it can also be synthesized from the catabolism of arginine, passing through the formation of _______

Answer 31

FIGLU Ornithine

Question 32

_____________- is a source of energy for muscle contraction

Answer 32

creatine phosphate

Question 33

inhibitory neutransmitter derivative of glutamic acid natural tranquilizer

Answer 33

GABA