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Block-6 Amino Disord

Block-6 Amino Metabolism Deficiency

QuestionAnswer
To what are amino acids converted when we are in the well-fed state? alpha-keto acids which go on to glycolysis, gluconeogenesis, and TCA
If an amino acid is converted to glucose it is called? If metabolized to acetyl-Co-A? Glucogenic Ketogenic
What 2 amino acids are exclusively Ketogenic? Lysine and Leucine
Two general symptoms of amino acid metabolism? retardation and failure to grow
Acetyl-CoA is converted into? Fatty Acid, used in TCA, Steriods/cholesterol
All newborns are screened for what amino acid disorder? PKU - phenylalanine Hydroxylase Deficiency
What physical traits and mental traits develop in PKU? Why? pale skin, blond hair, mental retardation, autism, loss motor control due to high levels of phenylalanine
What is the problem in PKU? deficient in phenyalanine hydroxylase to convert phenylalanine to tyrosine
Tyrosine is converted to another compound which can cause problems if not metabolised properly? Homogentisic Acid needs to be converted to maleyacetoacte by homogentisate oxidase (HO)
What symptoms appear with homogentisate Oxidase Deficiency? Name of disorder? Alcaptonuria, dark urine due to oxygenation when exposed to oxygen, dark pigment at joints (ochrononis)
Another disease (not PKU) is also screened for at birth. What are the enzyme responsible for it? Maple syrup urine disease, Isoleucine, Leucine, Valine (I Love Vermont)
What is the missing enzyme? problem if untreated? Branched-chain ketoacid dehydrogenase. If untreated infants become lethargic, fail to grow, Ketoacidosis: coma and death.
What amino acid is required as a cofactor for Branched-chain ketoacid d'hase? thiamine
What happens with proprionyl-CoA and Methymalonyl-CoA Mutase Deficiency? Like MSUD - results in neonatal keto, acidosis
What enzymes must be restricted in MSUD and proprionyl-CoA and Methymalonyl-CoA mutase defi... valine, methionine, isoluecine, threonine
What happens with a break down of cystathionine synthase? DVT, cardiovascular disease, homocysteine is highly reactive, binds LDL to form cholesterol fatty streaks/foam cells - die by age 25 of MI
Created by: El Diablo on 2010-02-23



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