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Basic Biochemistry

HY - Bill's Rate Limiting Enzymes - One-Liners

QuestionAnswer
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
Created by: HY90X