Most amino acids are generated inside the body
First ingested proteins enter the liver; which of the following are possible metabolic pathways for them?
Broken down for glucose
Sent from liver as branched chain amino acids
Converted urea and excreted
Broken down for the amino group
How are amino acids used by most organs (other than building proteins)?
Broken down for energy
To build proteins
To shunt metabolites around the blood
The carbon skeletons of amino acids can enter the intermediary metabolism at 7 different points to make energy
Amino acids assist in gluconeogesis by creating intermediates in the cycle that can then be used
Alanine can be converted to what?
Pyruvate + Glutamate
Ketoglutamate + Glutamate
OAA + Glutamate
Which amino acid can be converted to OAA/fumarate (and Glutamate) for the TCA cycle
Aspartate
Alanine
Glutamate
Glutamate is used to make Ketoglutamate for the TCA cycle and an NH4 for the urea cycle
Which aa is used to move excess NH3 from the peripheral tissues?
Arg- it can carry up to four extra NH3
Phe- it can carry up to four extra NH3
Asp- it can carry up to four extra NH3
Leu, Val and Ile are all examples of what?
Branched chain amino acids
Basic amino acids
Neutral amino acids
What is the use of branched chain amino acids?
Can be temporarily substituted for F.As in all tissues
Can be used to shuttle NH3 to the liver
Have carbon skeletons that can be attached to proteins
The inheritance of Maple Syrup Urine Disease is what kind?
Autosomal recessive
Autosomal dominant
Sex-Linked
In MSUD, the mutation is in Branched Chain K-Dehydrogenase, so branched chain amino acids cannot be broken down
How is MSUD treated?
Diet
Excess Glut & Ala
Excess Phe & Ser
