Amino Acids Part 2 Flashcards
(27 cards)
What major compounds derive nitrogen from amino acids?
Nitric oxide, hormones, neurotransmitters, nicotinamide, heme, creatin phosphate, and nucleotides
Where does the conversion of ammonia to urea take place?
Liver
FIRST STEP IN General scheme of amino acid catabolism
- remove amino group that is used to synthesize new nitrogen compounds or excreted as urea
SECOND STEP in general scheme of amino acid catabolism
incorporation of the carbon atoms into compounds that can enter the TCA cycle
Are the transaminase reactions reversible? Why
Yes, allows the inter-conversion between amino acids keeping their concentrations balanced
Ammonia is what to living organisms?
Ammonia is highly toxic & must be eliminated safely
What is the function of the UREA cycle
Converts:
- one molecule of free ammonia (from glutamic acid)
- one amino group from aspartate into urea and fumarate
Where does the free ammonia in the urea cycle come from?
Glutamic acid
where does the amino group in the urea cycle come from
Aspartate..which is converted into urea and fumarate
What is the connection between the urea cycle and the TCA cycle?
Through the product of fumarate during the urea cycle
What is used to transport nitrogen from peripheral tissues to the liver?
Alanine
What is alanine converted into, in the liver?
Alanine is converted into pyruvate with the nitrogen incorporated into urea
What facilitates the conversion of amino acids into glucose?
Glucose-alanine cycle
What is released by muscle into the blood?
Alanine & glutamine
How % does alanine & glutamine account for going into the blood?
50%
Where does the conversion of ammonia into carbamoyl phosphate occur?
Mitochondria
What conversion happens in the mitochondria?
Conversion of ammonia into carbamoyl phosphate
Urea Cycle activity
Can be lethel when effected. Several genetic defects affect the urea cycle
Whats good treatment if the urea cycle is affected?
Use of a compound that reacts with amino groups and remove them from the body
Glucogenic amino acids can be metabolized into what?
3 carbon molecules that can be used to synthesize glucose
What are ketogenic amino acids metabolized into?
2 or 4 carbon structures
Defects in amino acid metabolism
can become an inherited disease
Maple Syrup urine disease (MSUD)
inability to metabolize branch chain amino acids
What is Phenylkeonuria?
a deficiency of phenylalanine hydrooxylase that converts phenylalanine to tyrosine