amino acid catabolism Flashcards
(34 cards)
what % of energy production is from amino acid catabolism
10-15
how many catabolic pathways converge to form 5 products
20
what are the 5 products formed
acetyl CoA
A-Ketogluterate
succinyl CoA
fumarate
oxaloacetate
which of the 5 products cant be used to produce glucose
acetyl CoA
which of the products are assosiated with the TCA cycle
A-Ketogluterate
succinyl CoA
fumarate
oxaloacetate
which amino acids are used in energy production
glutamine- white blood cells and intestinal
epithelial cells- any dividing cell
branched chain amino acids- skeletal muscles
what are the features of glutamine
source of A-ketoglutarate
preferred fuel for some rapidly dividing cells, such as white blood cells and intestinal epithelial cells (spares glucose)
which branched chain amino acids are used as fuel in the skelteal muscles
valine, leucine and isoleucine
what are branched chain amino acids transanimated to produced
branched chain a-ketoacids
what substance is involved in maple syrup urine disease
branched chain A-ketoaciduria
describe maple syrup urine disease
genetic deficiency of branched chain A-ketoacid dehydrogenase
what happens if maple syrup urine disease is left untreated
seizures and eventually a coma
what does catabolism of phenylalanine and tyrosine generate
simultaneously produces acetyl CoA and fumarate
what do the substances produced from the catabolism of phenylalanine and tyrosine get used for
The TCA cycle
what happens due to phenylketonuria
the secondary pathway of phenylalanine metabolism is recruited
phenylalanine undergoes transamination with pyruvate to yield phenylpyruvate
what is elevated due to phenylketonuria
phenylpyruvate and phynelalaine in the blood and urine
what does phynylketonuria cause
impairs normal brain development and can cause tyrosine deficiency
what is methonine degraded to
succinyl-CoA
what stops the conversion of methionine to homocysteine
a lack of Vitamin Bs
what is wrong with having too much homocytesine
it is toxic and increases cardiovascular deficits
what does methionine act as
a methyl donor
what is folate involved in
the catabolism of glycine
what can glycine be used as
a methylene donor for biosynthetic reactions
where is the methylene group donated to
tetrahydrofolate