Block-6 Amino Metabolism Deficiency
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| 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 |
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Created by:
El Diablo
on 2010-02-23