Biochem Flashcards
2 carbons of the purine nitrogenous bases are derived from molecule(s)?
THF; Glycine\
2 nitrogens of the purine nitrogenous bases are derived from which amino acid?
Glutamine\
A defect in what enzyme in Tyrosine metabolism causes Albinism?
Tyrosinase\
A thiamine deficiency will impair which enzyme in the HMP shunt?
Transketolase\
Acetyl-CoA activates which enzyme in gluconeogenesis?
Pyruvate Carboxylase\
Aconitase forms which carbon structure in the TCA cycle?
Isocitrate\
Administration of which antimicrobial can cause a Niacin deficiency?
Isoniazid\
Alanine inhibits which enzyme in glycolysis?
Pyruvate kinase\
All-trans-retinoic acid (ATRA) is used a treatment for which condition?
Acute Promyelocytic Leukemia\
Alpha-Ketoglutarate Dehydrogenase forms which carbon structure in the TCA cycle?
Succinyl-CoA\
An overdose of which over-the-counter drug acts as an uncoupling agent of the inner mitochondrial membrane?
Aspirin\
At what age does Abetalipoproteinemia present?
Infancy\
At what stage does Classic Galactosemia present?
When started on breast milk\
At what stage does Hereditary Fructose Intolerance present?
When weaned from breast milk\
Azathioprine and 6-Mercaptopurine are activated by which enzyme?
HGPRT\
Azathioprine and 6-MP is metabolized by which enzyme?
Xanthine Oxidase\
Bacteria methylate which residue(s) on their DNA?
Adenine; Cytosine\
Biotin is a cofactor for which enzymes?
Pyruvate carboxylase; Acetyl-CoA-carboxylase; Propionyl-CoA carboxylase\
Biotin is a cofactor for which enzymes?
Pyruvate carboxylase; Acetyl-CoA-carboxylase; Propionyl-CoA carboxylase\
\f0 HDL?
Upregulation of PPAR-alpha\
Can the liver use ketone bodies for fuel?
No\
Cholesteryl esters are combinations of which molecules?
Cholesterol; Fatty acid\
Chronic excess of Vitamin A can cause enlargement of which organ?
Liver\
Citrate inhibits which enzyme in glycolysis?
PFK-1\
Citrate inhibits which enzyme in glycolysis?
PFK-1\
Citrate Synthase forms which carbon structure in the TCA cycle?
Citrate\
Complex I passes electrons to which molecule?
Coenzyme Q\
Complex III passes electrons to which molecule?
Cytochrome C\
Corneal vascularization is a symptom of which vitamin deficiency?
Vitamin B2 (Riboflavin)\
Creatinine is derived from which molecule?
Creatine\
Defect in Lipoprotein Lipase can cause which familial dyslipidemia?
Hyperchylomicronemia\
Deficiency in which enzyme in glycolysis can result in extravascular hemolysis of RBCs?
Pyruvate Kinase Deficiency\
Deficiency in which vitamin can cause a hemolytic anemia?
Vitamin E\
Deficiency in which vitamin can cause burning feet?
Vitamin B5\
Deficiency in which vitamin can cause Cheilosis?
Vitamin B2 (Riboflavin)\
Deficiency in which vitamin can cause tetany?
Vitamin D\
Diphyllobothrium latum causes a deficiency in which Vitamin?
Vitamin B12\
DNA methylation methylates which nitrogenous base(s)?
Cytosine Adenine\
Does Marasmus cause edema?
No\
Does Tay-Sachs disease have hepatosplenomegaly?
No\
Excess levels of which metabolite triggers production of ketone bodies?
Acetyl-CoA\
Ezetimibe blocks the absorption of which molecule in the small intestine?
Cholesterol\
F16BP activates which enzyme in glycolysis?
Pyruvate kinase\
FAD+ is derived from which Vitamin?
Vitamin B2 (Riboflavin)\
Foam cells are seen in which Lyosomal storage disease?
Niemann-Pick disease\
Folate is found in which type of foods?
Leafy green vegetables\
For which enzymes is thiamine a cofactor?
Pyruvate dehydrogenase; alpha-ketoglutarate dehydrogenase; alpha-ketoacid dehydrogenase; transketolase\
From which metabolic process is Ribose 5-phosphate formed which is used in purine synthesis?
HMP Shunt\
Fructose and glucose together make which disaccharide?
Sucrose\
Fructose is metabolized to which molecule, which can enter glycolysis?
Glyceraldehyde-3-phosphate\
Fumarase forms which carbon structure in the TCA cycle?
Malate\
GABA is a derivative of which amino acid?
Glutamate\
Galactokinase deficiency can cause which developmental defects?
Failure to track objects Lack of social smile, relating to vision loss due to cataracts\
Galactose and glucose together make which disaccharide?
Lactose\
Garlic-breath describes what type of poisoning?
Arsenic\
Globoid cells are seen in which lysosomal storage disease?
Krabbe disease\
Glycerol is converted to Glycerol-3-phosphate by what enzyme?
Glycerol Kinase\
Glycogen Phosphorylase releases which molecule?
Glucose-1-phosphate\
HDL is a repository for which apolipoproteins?
Apo C-II; Apo E\
High levels of Acetyl-CoA in the mitochondria drive which enzyme to metabolize Pyruvate?
Pyruvate Carboxylase\
High levels of orotic acid in the urine with hyperammonemia indicates which disease?
OTC deficiency\
Histones are rich in which amino acids?
Lysine; Arginine\
Homocysteine combines with which amino acid to create Cystathionine?
Serine\
Hormone sensitive lipase is activated by which hormone(s)?
Glucagon; Epinephrine\
\f0 TG?
Upregulation of Lipoprotein lipase\
\f0 TG?
Upregulate Lipoprotein Lipase\
How do Fibrates cause gallstones?
Inactivating 7alpha-hydroxylase\
How does Arsenic inhibit the Pyruvate Dehydrogenase complex?
Inhibits Lipoic Acid\
How does hyperammonemia inhibit the TCA cycle?
Depletion of alpha-KG\
How does Lactulose prevent absorption of NH4?
Lowers colonic pH and this promotes NH4+ formation over NH3, trapping it in the colon\
How does Vitamin C aid in the absorption of iron?
Conversion of Fe3+ to Fe2+\
Hunter syndrome involves a deficiency in which enzyme?
Iduronate-2-sulfatase\
Hurler syndrome involves a deficiency in which enzyme?
Alpha-L-iduronidase\
Hypohydrosis indicates which lysosomal storage disease?
Fabry disease\
Hypoxanthine is the nitrogenous base of which nucleotide?
IMP\
In amino acid breakdown, the amino group is initially transferred to which molecule in the muscle?
Alpha-ketoglutarate\
In Carcinoid Syndrome, there is a deficiency in which Vitamin?
Vitamin B3 (Niacin)\
In Cystinuria, there is decreased reabsorption of what molecules in the proximal renal tubule?
Cystine; Ornithine; Lysine; Arginine\
In Diabetic Ketoacidosis, low levels of which substance of the TCA cycle causes it to stall?
Oxaloacetate\
In Essential Fructosuria, which enzyme allows Fructose to enter glycolysis?
Hexokinase\
In fatty acid synthesis, which enzyme converts Citrate into Acetyl-CoA?
ATP-Citrate Lyase\
In gluconeogensis, where in the cell does FBPase-1 function?
Cytosol\
In gluconeogensis, where in the cell does Glucose-6-phosphatase function?
Endoplasmic reticulum\
In gluconeogensis, where in the cell does PEP Carboxykinase function?
Cytosol\
In gluconeogensis, where in the cell does Pyruvate Carboxylase function?
Mitochondria\
In glycogen hormonal regulation, what enzymes does glucagon phosphorylate?
Glycogen phosphorylase; Glycogen synthase\
In histone acetylation, an acetyl group is added to which amino acid residue?
Lysine\
In MCAD deficiency, what builds up in the blood?
Fatty acyl carnitine\
In pyrimidine synthesis, which amino acid is required for Carbamoyl Phosphate synthesis?
Glutamine\
In pyrimidine synthesis, which amino acid is required for Orotic Acid synthesis?
Aspartate\
In terms of cholesterol absorption, ACAT is found in which cell types?
Enterocytes; Hepatocytes\
In the kidney, URAT1 causes the absorption of which substance into the tubular cells?
Uric acid\
In the kidney, URAT1 causes the secretion of which substance into the tubular lumen?
Organic anions (such as Lactate)\
In the Malate shuttle for NADH entry, via which intermediate does Oxaloacetate leave the mitochondria?
Aspartate\
In the Malate shuttle, what does Oxaloacetate get converted to in order to leave the mitochondria?
Aspartate\
In what organelle is Glucose-6-Phosphatase active?
Endoplasmic Reticulum\
In which amino acid metabolism disorder must patients avoid Aspartame?
Phenylketonuria (PKU)\
In which conditions are there high levels of orotic acid in the urine?
Orotic aciduria; OTC deficiency\
In which familial dyslipidemia can patients get an MI before age 20?
Familial hypercholesterolemia\
In which Lysosomal Storage disease is there an accumulation of Ceramide Trihexose?
Fabry disease\
In which Lysosomal Storage disease is there an accumulation of Cerebroside sulfate?
Metachromatic leukodystrophy\
In which Lysosomal Storage disease is there an accumulation of Galactocerebroside?
Krabbe disease\
In which Lysosomal Storage disease is there an accumulation of Glucocerebroside?
Gaucher disease\
In which Lysosomal Storage disease is there an accumulation of GM2?
Tay-Sachs disease\
In which Lysosomal Storage disease is there an accumulation of Sphingomyelin?
Niemann-Pick disease\
In which Lysosomal Storage Diseases are there cherry red spots on the macula?
Tay Sachs disease; Niemann-Pick disease\
In which pulmonary condition can activation of Vitamin D occur independent of PCT cells in the kidney?
Sarcoidosis\
Is gluconeogenesis intact in Cori disease?
Yes\
Isocitrate Dehydrogenase forms which carbon structure in the TCA cycle?
Alpha-Ketoglutarate\
Ketone bodies are used as a source of energy by which organs?
Muscle; Heart; Brain\
Lack of which enzyme in purine breakdown can cause SCID?
Adenosine Deaminase\
Lactose is a combination of which two monosaccharides?
Galactose; Glucose\
LDH can be used to differentiate between which types of pleural effusions?
Transudate Exudate\
Lipoprotein lipase metabolizes which molecule from chylomicrons?
Triglycerides\
Lipoprotein Lipase requires which lipoprotein for activation?
Apo C-II\
Low levels of Acetyl-CoA in the mitochondria drive which enzyme to metabolize Pyruvate?
Pyruvate Dehydrogenase\
Lyosomes with onion skinning are seen in which lysosomal storage disease?
Tay-Sachs disease\
Malate Dehydrogenase forms which carbon structure in the TCA cycle?
Oxaloacetate\
MAO breaks down which molecule, which is then excreted as 5-HIAA?
Serotonin\
Massive consumption of raw egg whites can cause what vitamin deficiency?
Vitamin B7 (biotin)\
Melanin is derived from which amino acid?
Tyrosine\
Melatonin is derived from which amino acid?
Tryptophan\
Melatonin is synthesized from which neurotransmitter?
Serotonin\
Metabolism of the branched chain amino acids depends on which enzyme?
Alpha-ketoacid dehydrogenase\
Metyrosine inhibits which enzyme in catecholamine synthesis?
Tyrosine Hydroxylase\
Myopathy with Statins is increased when taking what other drugs?
Fibrates; Niacin\
N-acetylglutamate activates which enzyme in the Urea cycle?
CPS I\
Niacin can be synthesized by the body from which amino acid?
Tryptophan\
Niacin inhibits which enzyme in adipocytes?
Hormone sensitive lipase\
Niacin is derived from which amino acid?
Tryptophan\
Night blindness is a symptom of which vitamin deficiency?
Vitamin A\
Nitric Oxide Synthase uses which amino acid to create Nitric Oxide?
Arginine\
On which lipoproteins is C-II found?
Chylomicrons; VLDL; HDL\
On which molecule(s) is Apo B-48 found?
Chylomicrons; Chylomicron remnants\
Patients taking Levodopa/Carbidopa should avoid what Vitamin, in order to avoid the systemic effects of Dopamine?
Vitamin B6\
Patients with Classic Galactosemia must exclude what from their diet?
Galactose; Lactose\
Patients with Hartnup disease have a deficiency in which Vitamin?
Vitamin B3 (Niacin)\
Patients with Hereditary Fructose Intolerance will have a build-up of which molecule in Fructose metabolism?
Fructose-1-Phosphate\
Phenylketonuria (PKU) most commonly involves a deficiency in which enzyme?
Phenylalanine hydroxylase\
Pyruvate is transported into the mitochondria for which cellular processes?
TCA cycle; Gluconeogenesis (Pyruvate Carboxylase is in the mitochondria)\
SAM is synthesized from which amino acid?
Methionine\
SAM that loses Adenosine and a Methyl group becomes which molecule?
Homocysteine\
Statins increase the number of which type of receptor in the liver?
LDL receptors\
Subacute Combined Degeneration involves a deficiency in which Vitamin(s)?
Vitamin B12\
Succinate Dehydrogenase forms which carbon structure in the TCA cycle?
Fumarate\
Succinyl-CoA Synthetase forms which carbon structure in the TCA cycle?
Succinate\
Sucrose is a combination of which two monosaccharides?
Fructose; Glucose\
Synthesis of Niacin from its amino acid precursor requires which cofactor(s)?
Vitamin B2; Vitamin B6\
The carbons and nitrogens in Urea are derived from which molecules?
NH3; CO2; Aspartate\
The liver secretes which lipoprotein(s) to deliver cholesterol and triglycerides to the body?
VLDL; HDL\
The Pyruvate Dehydrogenase complex is located in which part of the cell?
Mitochondria\
The Pyruvate Dehydrogenase complex requires what cofactors?
Thiamine pyrophosphate (B1); FAD (B2); NAD (B3); Coenzyme A (B5); Lipoic acid\
Thiamine deficiency causes which type of cardiomyopathy?
Dilated cardiomyopathy\
Thiamine is a cofactor for which enzyme of the HMP shunt?
Transketolase\
Thiamine is a cofactor for which enzyme(s) of the TCA cycle?
Alpha-ketoglutarate dehydrogenase\
Thiamine is a cofactor for which enzymes?
Pyruvate dehydrogenase; Alpha-ketoglutarate dehydrogenase; alpha-ketoacid dehydrogenase; Transketolase\
Thyroid hormone is derived from which amino acid?
Tyrosine\
Treatment of Maple Syrup Urine Disease involve supplementation with which vitamin?
Thiamine\
Tyrosine is degraded into what component of the TCA cycle?
Fumarate\
UMP inhibits what enzyme in pyrimidine synthesis?
Carbamoyl Phosphate Synthetase II\
Uridine is the treatment for what defect in pyrimidine synthesis?
Orotic aciduria\
Uridine is the treatment for which disease of Pyrimidine Synthesis?
Orotic Aciduria\
Vitamin A is used to treat which infectious disease?
Measles\
Vitamin B12 contains which metal element?
Cobalt\
Vitamin C is an ancillary treatment for which hemoglobin modification condition?
Methemoglobinemia\
What accumulates in Hunter syndrome?
Heparan sulfate; Dermatan sulfate\
What accumulates in Hurler syndrome?
Heparan sulfate; Dermatan sulfate\
What activates Fructose-1,6-bisphosphatase?
Citrate\
What activates Isocitrate Dehydrogenase?
ADP\
What activates Pyruvate Kinase?
Fructose 1,6 BP\
What aids the absorption of Iron via conversion of Fe3+ to Fe2+?
Vitamin C\
What anti-malarial drugs can precipitate a G6PD deficiency crisis?
Primaquine Quinidine\
What apolipoprotein is required on Chylomicrons for secretion from enterocytes?
Apo B-48\
What apolipoproteins are found on VLDL?
Apo B-100; Apo C-II; Apo E\
What apolipoproteins are present on Chylomicron remnants?
E; B-48\
What apolipoproteins are present on Chylomicrons?
B-48; C-II; E\
What apolipoproteins are present on HDL?
A-I; C-II; E\
What apolipoproteins are present on IDL?
E; B-100\
What apolipoproteins are present on LDL?
B-100\
What apolipoproteins are present on VLDL?
B-100; C-II; E\
What apoproteins are on HDL?
A-I; C-II; Apo E\
What are 3 uncoupling agents of oxidative phosphorylation?
2,4, DNP; Aspirin; Thermogenin in brown fat\
What are examples of purine rich foods, which are more likely to precipitate an acute gout attack?
Red meat; Seafood\
What are the acidic amino acids?
Aspartic acid; Glutaminc acid\
What are the activators of Glycogen synthase?
Glucose-6-phosphate; Insulin; Cortisol\
What are the activators of PFK-1?
AMP; Fructose-2,6-bisphosphate\
What are the adverse effects of bile acid resins?
GI upset; Decreased absorption of fat soluble vitamins/drugs\
What are the adverse effects of Fibrates?
Myopathy; Cholesterol gallstones\
What are the adverse effects of HMG-CoA reductase inhibitors?
Hepatotoxicity; Myopathy\
What are the adverse effects of Niacin?
Facial flushing; Hyperglycemia; Hyperuricemia\
What are the antioxidant vitamins?
Vitamin A; Vitamin C; Vitamin E\
What are the basic amino acids?
Histidine; Lysine; Arginine\
What are the branched chain amino acids?
Leucine; Isoleucine; Valine\
What are the branched chain amino acids?
Leucine; Isoleucine; Valine\
What are the cardiac findings in Carcinoid Syndrome?
Right-sided valvular heart disease\
What are the cardiovascular effects of Homocystinuria?
Thrombosis/Atherosclerosis Stroke/MI\
What are the classic patients who have a folate deficiency?
Alcoholics; Pregnant women\
What are the clinical findings in Pyruvate dehydrogenase complex deficiency?
Neurologic defects; Lactic acidosis;
What are the clinical findings in the skin of patients with Vitamin A deficiency?
Dry, scaly skin (xerosis cutis)\
What are the clinical indications for Hydroxyurea?
Sickle cell anemia; Myeloproliferative disorders\
