AA Metabolism I/II

Question 1

What is a key transport form of Nitrogen?

Answer 1

Glutamine

Question 2

Most Amino groups harvested from AA are converted to ______ though the ______ cycle.

Answer 2

Urea

Urea

Question 3

Purpose of Alanine aminotranferase?

Answer 3

Catalyzes the transfer of of the amino group of ALANINE to Alpha-ketoglutarate

Question 4

What coenzyme plays a key role in AA degradation?

Answer 4

Pyridoxal Phosphate

Question 5

What happens after Citrulline leaves the Mitochondrial matrix?

Answer 5

It condenses with ASPARTATE to form Argininosuccinate

Question 6

AA degraded to Pyruvate, Alpha-Ketoglutarate, Succinyl CoA, Fumarate, or OAA are termed _____ ?

Answer 6

Glucogenic

Question 7

Carbamoyl Phosphate reacts with ____ to begin the Urea Cycle?

Answer 7

Ornithine

Question 8

What enzyme catalyzes the formation of ALA?

Where is it the enzyme present?

Answer 8

ALA Synthase

Present in the Mitochondria

Question 9

What happens after Urea is produced from the Hydrolysis of Arginine?

Answer 9

It gets Excreted

Question 10

Citrulline is Produced where?

Does it stay there?

Answer 10

In the Mitochondrial matrix

NO, it gets transported into the cytoplasm

Question 11

What is the first step in the Biosynthesis of Porphyrins?

Answer 11

The Condensation of Glycine and Succinyl CoA to form Alpha-Aminolevulinate (ALA)

Question 12

Production of Nitric Oxide

Precursor and Enzyme

Answer 12

Produced from Arginine

Catalyzed from NO Synthase

Question 13

Fxn of ·NO

Answer 13

Vasodilation

Question 14

Glutathione Peroxidase fxn?

Answer 14

The enzyme that catalyzes the peroxide detoxification.

Question 15

What AA is a precursor of Serotonin?

Answer 15

Tryptophan

Question 16

Alpha-Amino groups are converted to ______ by the oxidative deamination of ______?

Answer 16

Ammonium

GlutaMATE

Question 17

What AA is a precursor of Epinephrine?

Answer 17

Tyrosine

Question 18

Carbamoyl Phosphate if produced what two reactants?

Answer 18

CO2 + NH4+

Or

HCO3- + NH3

Question 19

What AA is the precursor of Histamine?

Answer 19

Histidine

Question 20

What enzyme synthesizes the formation of Carbamoyl Phosphate?

Answer 20

Carbamoyl Phosphate Synthase I

Question 21

AA degraded to ACoA or Acetoacetyl CoA are termed _____ because they give rise to _______?

Answer 21

Ketogenic

Ketone Bodies or FA

Question 22

What enzymes catalyze the transfer of an alpha-amino group to what kind of acid?

Answer 22

Aminotransferases
(Transaminases)

Alpha-KETOacid

Question 23

Aside from Arginine, what are required for the synthesis for ·NO?

Answer 23

NADPH and O2

Question 24

Carbamoyl Phosphate reacts with Ornithine to produce _______?

The rxn is catalyzed by what enzyme?

Answer 24

Citrulline

Ornithine Transcarbamoylase

Question 25

The nitrogen atom from Glutamate is converted into _____ by the enzyme _____?

Answer 25

Free Ammonium Glutamate Dehydrogenase

Question 26

Why is Glutamate Dehydrogenase so unusual?

Answer 26

Because it can utilize either NAD+ or NADP+

Question 27

Alanine + Alpha Ketoglutarate yields what products?

Answer 27

PYRUVATE and Glutamate Think “PAL”

Question 28

What AA is a precursor to Sphingosine?

Answer 28

Serine

Question 29

Reaction to produce Ammonium

Answer 29

AA ——> Glutamate Glutamate ——> Ammonium

Question 30

What element is associated with Glutathione Peroxidase?

Answer 30

Selenium

Question 31

Components of Glutathione?

Answer 31

Gamma-Glutamate + Cysteine + Glycine (GCG)

Question 32

Glutamate Dehydrogenase is inhibited by _____ and is stimulated by ______?

Answer 32

Inhibited by GTP Stimulated by ADP

Question 33

Urea is synthesized from what two Nitrogen sources?

Answer 33

Aspartate & Free NH4+

Question 34

Aspartate + Alpha Ketoglutarate yields what products?

Answer 34

Oxaloacetate and Glutamate Think “OX ASs”

Question 35

What is the rxn of the enzyme Argininosuccinase?

Answer 35

Aka Argininosuccinate Lyase Cleaves AS into ARGININE and FUMARATE - Fumarate leave the cycle

Question 36

What AA is a precursor of the Nicotinamide unit of NAD+?

Answer 36

Tryptophan

Question 37

What AA is a precursor of Thyroxine?

Answer 37

Tyrosine

Question 38

What is the purpose of the production of Fumarate?

Answer 38

Precursor for Glucose synthesis.

Question 39

What is the Committed reaction of the Urea Cycle?

Answer 39

The formation of Carbamoyl Phosphate

Question 40

Purpose of Aspartate Aminotransferase?

Answer 40

Catalyzes the transfer of the amino group of Aspartate to Alpha-Ketoglutarate

Question 41

The purpose of the enzyme Arginase?

Answer 41

To hydrolyze Arginine to generate UREA and Ornithine

Question 42

The carbon sources of AA come from what Pathaways?

Answer 42

1. Glycolysis 2. PPP 3. TCA

Question 43

Role of Glutathione

Answer 43

Serves as a sulfhydryl buffer and an Antioxidant

Question 44

The production of Argininosuccinate is catalyzed by what enzyme? What is the energy source?

Answer 44

Argininosuccinate Synthase ATP ——> AMP

Question 45

All defects in the Urea Cycle can lead to what disease?

Answer 45

Hyperammonemia | - elevated level of NH4+ in the BLOOD

Question 46

Degradation of Heme What energy source is required?

Answer 46

Heme ——>BiliVERDIN ———> BiliRUBIN NADPH is used for both rxns

Question 47

What happens after Ornithine is produced from the Hydrolysis of Arginine?

Answer 47

It gets shuttled back into the Mitochondrial matrix