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Basic Biochemistry
HY - Bill's Rate Limiting Enzymes - One-Liners
| Question | Answer |
|---|---|
| RLE of Glycolysis | Phosphofructokinase - I |
| RLE of Gluconeogenesis | Fructose 1,6-bisphosphatase |
| RLE of TCA cycle | Isocitrate dehydrogenase |
| RLE of glycogen sythesis | Glycogen Synthase |
| RLE of glycogenolysis | Glycogen Phosporylase |
| RLE of HMP shunt (aka the Hexose monophosphate shunt or the pentose phosphate pathway) | Glucose-6-phosphate dehydrogenase |
| RLE of pyrimidine synthesis | CPS II |
| RLE of Purine sythesis | Glutamine-PRPP-amidotransferase |
| RLE of Urea cycle | CPS I |
| RLE of Fatty acid sythesis | Acetyl-CoA carboxylase |
| RLE of fatty acid oxidation | Carnitine acyltransferase I |
| RLE of Ketogenesis | HMG-CoA synthase |
| RLE of Cholesterol sythesis | HMG-CoA reductase |
| What processes are NADPH used in? | Anabolic processes, Respiratory burst, P-450, and Glutathion reductase |
| The following molecules will have an increase or decrease in the rate of glycolysis? 1) ATP 2) AMP 3) Citrate 4) Fructose-2,6-Bisphosphate | 1) Decreases because you have lots of energy 2) Increases because you have no energy 3) Decreases because the TCA cycle needs to catch up 4) Increases because you need to further drive glycolysis |
| What cofactors are required for pyruvate dehydrogenase and α-ketoglutarate dehydrogenase? | Thiamine, FAD (riboflavin), NAD (Niacin), CoA (pantothenate) and Lipoic acid |
| Increased levels of which molecules with increase the rate of pyruvate dehydrogenase? | Increased levels of NAD+/NADH, ADP, and Ca+ **high levels of the above suggests you need energy** |
| What other molecules can pyruvate be made into? | Glucose, Lactate, Acetyl-CoA, Oxaloacetate, and Alanine |
| What is the purpose of the Cori Cycle? | Allows lactate to be made into glucose! |
| What is the purpose of the HMP shunt? | To make NADPH for glutathione protection and ribose |
| What disease is caused by failure to make NADPH and protect against free radical damage? | Glucose-6-phosphate dehydrogenase deficiency |
| What enzyme is deficient in Fuctose Intolerance? | Aldolase B |
| What enzyme is deficient in Essential Fructosuria? | Fructokinase |
| What is the difference between the two above disorders? | Essential Frucosuria is benign (Fructose intolerance has hypoglycemia, jaundice, cirrhosis and vomiting) |
| What enzyme is deficient in Classic Galactosemia? | galactose-1-phosphate uridyltransferase |
| What enzyme is deficient in Galactokinase deficiency? | galactokinase |
| Symptoms of classic galactosemia? | failure to thrive, jaundice, cataracts, hepatomegaly, MR |
| Symptoms of galactokinase deficiency? | mild condition, galactose in urine, cataracts, no social smile |
| What is the most common Urea cycle disorder? | Ornithine transcarbamoylase deficiency (OTC) |
| Symptoms of OTC deficiency? | High Orotic acid, decreased BUN, hyperammonemia |
| What can be made from Phenylalanine? | Tyrosine, DOPA, Dopamine, Thyroxine, Melanin, NE, Epi |
| What can be made from Tryptophan? | Serotonin, Melatonin, Niacin |
| What can be made from Histidine? | Histamine |
| What can be made from Glycine? | Porphyrin, Heme |
| What can be made from Arginine? | Creatine, Urea, and Nitric Oxide |
| What can be made from Glutamate? | GABA, and glutathione |
| What is the cause of Phenylketonuria? | Lack of Phenylalanine Hydroxylase or BH4 |
| What is the cause of alkaptonuria (ochronosis)? | lack of homogentisic acid oxidase |
| What is the cause of albinism? | Lack of tyrosinase or tyrosine transporters |
| What causes homocystinuria? | Lack of Cystathione synthase low affinity of above for pyridoxal phosphate Homocysteine methyltransferase deficiency |
| What are the symptoms of homocystinuria? | Mental retardation, osteoporosis, tall stature, kyphosis lens subluxation, atherosclerosis |
| What is the cause of cystinuria? | Defect in renal tubular amino acid transporter |
| What are the symptoms of cystinuria? | Cystine Kidney stones (Rx. Acetazolamide to increase pH) |
| What is the cause of maple syrup urine disease? | Can't degraded branched chain A.As (Ile, Leu, Val) |
| Which disease results in loss of tryptophan in the urine and leads to pellagra? | Hartnup disease |
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HY90X
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