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Biochemistry
FA complete review part 2
| Question | Answer |
|---|---|
| What complex links glycolysis and TCA cycle? | Pyruvate dehydrogenase complex |
| Which state, fed or fasting, activates the Pyruvate dehydrogenase complex? | Fed state |
| Which enzyme has a similar role to Pyruvate dehydrogenase complex? | a-ketoglutarate dehydrogenase |
| What are the 5 cofactors required by Pyruvate dehydrogenase? | 1. Thiamine pyrophosphate (B1) 2. Lipoic acid 3. CoA 4. FAD 5. NAD+ |
| What 3 conditions activate the Pyruvate Dehydrogenase complex ? | INCREASED: - NAD+/NADH ratio - ADP - Ca2+ |
| What is the classical description of Arsenic poisoning breath? | Garlic |
| Arsenic inhibits with Pyruvate dehydrogenase complex cofactor? | Lipoic acid |
| What is the mode of inheritance of Pyruvate dehydrogenase deficiency? | X-linked |
| What is caused by Pyruvate dehydrogenase complex deficiency? | Buildup of pyruvate that gets shunted to 1) lactate via LDH, and 2) alanine via ALT |
| What serum abnormality is seen since infancy in Pyruvate dehydrogenase complex deficiency? | Increased serum alanine |
| What is the treatment of Pyruvate dehydrogenase complex deficiency? | Increase intake of ketogenic nutrients (lysine and leucine rich products) |
| Neurologic defects + lactic acidosis + increased serum alanine in a 2 year old patient. Dx? | Pyruvate dehydrogenase complex deficiency |
| What are the four destinations of Pyruvate metabolism? | Alanine, Oxaloacetate, Acetyl-CoA, and Lactate |
| Which are the two fates of Pyruvate that occur in the cytosol? | Alanine (Cahill cycle) and Lactae (Cori cycle) |
| Which ar the two mitochondrial fates of Pyruvate? | Oxaloacetate and Acetyl -CoA |
| What are uses or purposes of Oxaloacetate? | 1. Replenish TCA cycle 2. Used in Gluconeogenesis |
| What enzyme is used for the transition of Glycolysis to the TCA cycle? | Pyruvate dehydrogenase |
| The production of Lactate, signals the: | End of anaerobic glycolysis |
| Lactate production is the major pathway of ATP production in which tissues? | RBCs, WBCs, kidney medulla, lens, testes, and cornea |
| What cofactor is used by Lactic acid dehydrogenase? | B3 |
| What enzyme is required to by Pyruvate in order to concert into Acetyl-CoA? | Pyruvate dehydrogenase (PDH) |
| Which enzyme is PC, and is used for? | Pyruvate carboxylase; used to convert Pyruvate into Oxaloacetate |
| How many carbon dioxide molecules are produced by the TCA cycle? | 2 |
| What is another name for the TCA cycle? | Krebs cycle |
| Which TCA cycle enzyme shares the same cofactors as Pyruvate dehydrogenase complex? | alpha-Ketoglutarate dehydrogenase complex |
| What are the 3 irreversible TCA cycle enzymes? | Citrate synthase, Isocitrate dehydrogenase, and a-KG dehydrogenase |
| Enzyme used in Complex II of the ETC? | Succinate dehydrogenase |
| Common inhibitors of ETC Complex IV? | Cyanide, CO, and Azide |
| Cyanide poisoning causes a decrease in aerobic ATP production due to: | Inhibition of Complex IV in the ETC |
| Which drug inhibits Complex I? | Rotenone |
| Complex III in the ETC is inhibited by ______________________. | Antimycin A |
| Oligomycin inhibits or halts ATP production by : | Inhibition of Complex V in the ETC |
| Which type of Oxidative phosphorylation agents cause a decrease in proton gradient in the ETC? | Electron transport inhibitors and Uncoupling agents |
| What are common Electron transport inhibitors? | Rotenone, Antimycin A, Cyanide, carbon monoxide, and azide |
| 2,4-Dinitrophenol is an __________________, used illicity to loss weight. | Uncoupling agent |
| Aspirin and Thermogenin are both ________________ agents. | Uncoupling |
| What is the most common ATP synthase inhibitor? | Oligomycin |
| What is the primary function of Gluconeogenesis? | Maintain euglycemia during fasting states |
| What is caused by a deficiency in key gluconeogenic enzymes? | Hypoglycemia |
| Why do even-chain fatty acids cannot produce new glucose? | They yield only one acetyl-CoA equivalent |
| Why do only odd-chain fatty acids undergo gluconeogenesis, and serve as glucose source? | These yield 1 propionyl-CoA |
| Another name for the HMP shunt? | Pentose Phosphate Pathway |
| Which product is primarily provided by the HMP shunt? | NADPH |
| How much ATP is used or produced by the HMP shunt? | None |
| What are the two main products of the HMP shunt? | 1. NADPH 2. Ribose for nucleotide synthesis |
| Which sites have high HMP shunt activity? | Lactating mammary glands, liver, adrenal cortex, and RBCs |
| What are the two divisions of the HMP shunt? | Oxidative (irreversible) and Nonoxidative (reversible) |
| Which is the main enzyme involved in the Oxidative reaction of the HMP shunt? | Glucose-6-P dehydrogenase |
| What are the two enzymes involved in non-oxidative part of the HMP shunt? | Phosphopentose isomerase, and Transketolase |
| What vitamin is required, as cofactor, in the nonoxidative reaction of the HMP shunt? | Vitamin B1 |
| Which part or reaction produces Ribose-5-P of the HMP shunt? | Nonoxidative |
| Carbon dioxide, 2 NADPH, and Ribulose-5-P are products of: | Oxidative reaction of the HMP shunt |
| What enzyme is needed to keep glutathione reduced? | NADPH |
| The reduced form of glutathione functions by: | Detoxification of free radicals and peroxides |
| What enzyme deficiency can, most commonly, cause a deficiency in NADPH? | G6PD |
| What is the most common human enzyme deficeincy? | G6PD deficiency |
| G6PD deficiency is an _________________ disorder. | X-linked recessive |
| Which population is at higher risk of developing G6PD deficiency? | African Americans |
| What is a inadvertent benefit of G6PD deficiency? | Increase malarial resistance |
| What kind of anemia is produced in G6PD deficiency? | Hemolytic anemia due to poor RBC defense against oxidizing agents |
| What are some oxidizing agents that precipitate G6PD deficiency? | Fava beans, sulfonamides, nitrofurantoin, primaquine/chloroquine, antituberculosis drugs |
| Which condition is seen with Heinz bodies and Bite cells? | G6PD deficiency |
| What are Heinz bodies? | Denatured globin chains precipitate within RBCs due to oxidative stress |
| What are Bite cells? | Result form the phagocytic removal of Heinz bodies by splenic macrophages |
| In a patient with Essential Fructosuria, which becomes the primary pathways for converting fructose into fructose-6-phosphate? | Hexokinase |
| What are the symptoms of essential Fructosuria? | Fructose in blood and urine |
| Which fructose metabolism is most severe? | Hereditary fructose intolerance due to Aldolase B deficiency |
| Both fructose metabolism disorders are of ______________________ inheritance. | Autosomal recessive |
| What metabolic processes are inhibited in Fructose intolerance? | Glycogenolysis and Gluconeogenesis |
| Which is a common vignette scenario of a person with Fructose intolerance? | Acute development of hypoglycemia following consumption of fruit, juice, or honey. |
| What is the result of a urine dipstick test in Fructose intolerance? | Negative |
| Which is the defective or absent enzyme of Fructose intolerance? | Aldolase B |
| What are the clinical manifestations of Aldolase B deficiency? | Hypoglycemia, jaundice, cirrhosis, and vomiting. |
| Which Galactose metabolism disorder is most severe? | Classic Galactosemia |
| Hereditary deficiency of Galactokinase. Dx? | Galactokinase deficiency |
| What are the most common clinical symptoms of Galactokinase deficiency? | Galactose in blood and urine, infantile cataracts, and possible failure to track objects or develop social smile. |
| Classical Galactosemia is due to: | Absence of galactose-1-phosphate uridyltransferase |
| What is accumulated in the lens of the eye in a patient with Classic Galactosemia? | Galactitol |
| What are the classical symptoms and presentation of a infant with Classic Galactosemia? | Failure to thrive, jaundice, hepatomegaly, infantile cataracts, intellectual disability, and predisposition to E. coli sepsis in neonates. |
| What kind of sepsis is possibly seen in a neonate with Classic Galactosemia? | E. coli |
| Which advance condition may lead to phosphate depletion? | Classic Galactosemia |
| What is an alternate form of trapping glucose in the cell? | Sorbitol |
| What enzyme is used to convert glucose into sorbitol? | Aldose reductase |
| What is the alcohol counterpart of glucose? | Sorbitol |
| What are some manifestations of osmotic damage due to sorbitol accumulation? | Cataracts, retinopathy, and peripheral neuropathy |
| Enzyme that converts Sorbitol into Fructose? | Sorbitol dehydrogenase |
| Which enzyme converts Glucose ----> Sorbitol? | Aldose reductase |
| Where does Lactase work? | Intestinal brush border to digest lactose |
| Lactose breaks down lactose into ______ and ___________. | Glucose and Galactose |
| What is the primary reason of Lactase deficiency? | Absence of lactase-persistence allele |
| Loss of intestinal brush border due to GI infection, is the secondary cause of _____________________ deficiency. | Lactase |
| What are the corresponding labs of stool and breath analysis of a patient with Lactose intolerance? | Decreased pH and breath shows hydrogen content with lactose hydrogen breath test |
| Patient with bloating, caprms, flatulence, and osmotic diarrhea after eating ice cream. Dx? | Lactase deficiency |
| Which isoform of amino acids is found in proteins? | L-amino acids |
| Mnemonic for Essential amino acids | PVT TIM HaLL |
| Which are all the essential amino acids? | Phenylalanine, Valine, Tyrosine, Threonine, Isoleucine, Mthione, Histidine, Leucine, and Lysine |
| Which are the the Essential purely Glucogenic amino acids? | Methionine, Histidine, and valine |
| Purely Ketogenic amino acids? | Leucine and Lysine |
| Isoleucine, Phenylalanine, Threonine, and Tyrosine are: | Both, Glucogenic and Ketogenic amino acids |
| Which are the 2 acidic amino acids? | Aspartic acid and Glutamic acid |
| Which are the 3 basic amino acids? | Arginine, histidine, and lysine |
| Essential amino acids are categorized into which categories? | Glucogenic, Ketogenic , and Mixed (ketogenic/glucogenic) |
| Which is the most basic amino acid? | Arginine |
| Which two basic amino acids are required in periods of growth? | Arginine and histidine |
| What is the charge of acidic amino acids at body pH? | Negative |
| What cycle produces common metabolites of amino acids such as Pyruvate and Acetyl-CoA, which serve as metabolic fuels? | Urea cycle |
| Excess urea produced in Urea cycle is excreted via the _____. | Kidneys |
| The Cahill cycle connects the _____ with the _______, in order to secrete urea (NH3). | Muscle to the liver |
| What is a common hereditary form of Hyperammonemia? | Urea cycle enzyme deficiencies |
| What happens in the CNS in cases of excess NH3? | NH3 deprest glutamate (GABA) in CNS and a-Ketoglutarate leading to inhibition of TCA cycle. |
| What are clinical manifestation of Ammonia accumulation? | Flapping tremor (asterixis), slurring of speech, somnolence, vomiting, cerebral edema, and blurring of vision |
| What medication may be given to reduced amononal levels? | 1. Lactulose 2. Rifaximin and Neomycin 3. Benzoate, phenylacetate |
| What is the MOA of Lactulose? | Acidify the GI tract and trap NH4+ for excretion |
| What is the reason of RIfaximin or Neomycin use to reduce ammonia levels? | Decrease colonic ammoniagenic bacteria |
| What is the most common urea cycle disorder? | Ornithine transcarbamylase deficiency |
| Which condition is due to the body's inability to eliminate ammonia? | Ornithine transcarbamylase deficiency |
| What are the common findings of Ornithine transcarbamylase deficiency? | - Increased orotic acid in blood and urine - Decreased BUN - Symptoms of Hyperammonemia |
| What is a key clinical finding that differentiates Orotic aciduria from Ornithine Transcarbamylase deficiency? | Ornithine transcarbamylase deficiency does NOT present with megaloblastic anemia |
| What is the inheritance mode of Ornithine transcarbamylase deficiency? | X-linked recessive |
| What are the derivatives of Tryptophan? | NIacin, Serotonin, and Melatonin |
| What are the derivatives of Glycine? | Porphyrin --> Heme |
| GABA is made from which amino acid? | Glutamate |
| What are the 3 main derivatives of Arginine? | Creatine, Urea, and Nitric Oxide |
| Phenylalanine + Tetrahydrobiopterin (BH4) = | Tyrosine |
| Dopamine needs what vitamin in order to produce or convert into Norepinephrine? | Vitamin C |
| NE + SAM ---------> | Epinephrine |
| Phenylalanine hydroxylase deficiency. Dx? | PKU |
| What enzyme is deficient in PKU? | Phenylalanine hydroxylase |
| PKU may be due to deficiency in which enzyme and cofactor? | 1. Phenylalanine hydroxylase 2. BH4 |
| What enzyme deficiency leads to Alkaptonuria? | Homogentisate oxidase |
| Decreased levels of Maleyl Acetoacetic acid most likely is due to ____________________, which has a deficiency in _________________. | Alkaptonuria; Homogentisate oxidase |
| Albinism is due to deficiency in which enzyme? | Tyrosinase |
| PKU is seen with elevated levels of _________________. | Phenylalanine |
| Malignant PKU is due to deficiency in _________________. | BH4 |
| How soon is can PKU be diagnosed? | 2-3 days after birth |
| How are increased levels of Phenylalanine represented in urine? | Excess phenyl ketones |
| What are clinical findings of Phenylketonuria (PKU)? | Intellectual disability, growth retardation, seizures, fair complexion, eczema, and musty body odor |
| Musty body odor. Dx? | PKU |
| What is increased in the diet of a PKU patient? | Increased consumption or tyrosine |
| What food product do PKU patients must avoid at all costs from ingestion? | Artificial sweetener aspartame, since it contains phenylalanine |
| What is the MCC of Maternal PKU? | Lack of proper dietary therapy during pregnancy |
| What the MCC of Maple Syrup urine disease? | Blocked degradation of branched amino acids due to decreased branched-chain a-ketoacid dehydrogenase (B1) |
| What are the branched amino acids affected in Maple Syrup urine disease? | Isoleucine, Leucine, and Valine |
| What is the characteristic smell or odor of urine a patient with maple syrup urine disease? | Maple syrup/ or burnt sugar |
| What is the most elevated a-ketoacid in MSUD? | Leucine |
| What is the mode of inheritance of MSUD? | Autosomal recessive |
| What are the findings seen with Alkaptonuria? | Bluish-black connective tissue, ear cartilage, and sclerae |
| Urine turns black after prolonged exposure to air. Dx? | Alkaptonuria |
| Why is Alkaptonuria presented with debilitating arthralgias? | Homogentisic acid toxic to cartilage |
| What is the mode of inheritance of all types of Homocystinuria? | Autosomal recessive |
| What are the 3 types/ causes of Homocystinuria? | 1. Cystathionine synthase deficiency 2. Decreased affinity of cystathionine synthase for pyridoxal phosphate 3. Methionine synthase deficiency |
| What are the clinical manifestations of Homocystinuria? | - Severely elevated levels of Homocysteine in urine - Osteoporosis - Marfanoid habitus - Lens subluxation (down and in) - CV effects (stroke and MI) - Kyphosis - Intellectual disability |
| What are the Cardiovascular effects seen in Homocystinuria? | Thrombosis and atherosclerosis ---> Stroke and MI |
| What vitamin is required for Methionine synthase to produce Methionine? | Vitamin B12 |
| Homocysteine + Serine require Vitamin ______ and _________________ to produce cystathionine and later Cysteine. | Vitamin B6; Cystathionine synthase |
| Hereditary defect of renal PCT and intestinal amino acid transporter that prevents reabsorption of Cysteine, Ornithine, Lysine, and Arginine. Dx? | Cystinuria |
| What test is diagnostic for Cystinuria? | Urinary cyanide-nitroprusside test |
| What amino acids are not reabsorbed n Cystinuria patients? | Cysteine, Ornithine, Lysine, and Arginine |
| Hexagonal cystine stones. Dx? | Cystinuria |
| How is Cystine formed? | 2 Cysteines connected by a disulfide bond |
| What diuretics can be used to alkalinize urine in Cystinuria patients? | Potassium citrate, acetazolamide |
| What stain is useful to identify glycogen storage diseases? | Periodic acid-Schiff stain |
| What is the name of the Type I glycogen storage disease? | Von Gierke disease |
| Name of type II glycogen storage disease? | Pompe disease |
| Cori disease if type ______ (glycogen storage disease). | III |
| What is the name of Type V Glycogen Storage disease? | McArdle disease |
| Deficient in Glucose-6-Phosphatase. Dx? | Von Gierke disease |
| What metabolic processes are inhibited in Von Gierke disease? | Gluconeogenesis and Glycogenolysis |
| What the characteristics findings of von Gierke disease? | - Severe fasting hypoglycemia, - Greatly Glycogen in liver and kidneys - Blood lactate - Gout due to increased uric acid - Hepatomegaly and renomegaly |
| Clinical findings of Pompe disease: | Cardiomegaly, HCM, hypotonia, exercise intolerance and early death. |
| What enzyme is deficient in Pompe disease? | Lysosomal acid a-1,4-glucosidase |
| What is a milder form of von Gierke disease? | Cori disease |
| Deficient enzyme of Cori disease? | Debranching enzyme (a-1,6-glucosidase) |
| Deficient skeletal muscle glycogen phosphorylase. Dx? | McArdle disease |
| What is another name of Skeletal muscle glycogen phosphorylase? | Myophosphorylase |
| What is a Hallmark feature of McArdle disease? | Flat venous lactate curve with normal rise in ammonia levels during exercise |
| How is blood serum affected in Type V Glycogen storage disease? | Unaffected |
| What causes the muscle pain in McArdle disease? | Increased glycogen in muscle, but the muscle cannot break it down --> painful Muscle cramps, Myoglobinuria (red urine), with strenuous exercise, and arrhythmia form electrolyte abnormalities. |
| What is Glycogen? | Stored form of glucose in the liver |
| What is Glucagon? | Secreted from alpha cells of the pancreas |
| Glucagon breaks into ___________ and ____________ in the liver. | Glucose and Glycogen |
| What are the main two divisions of Lysosomal Storage diseases? | Sphingolipidoses and Mucopolysaccharidoses |
| What are the two Mucopolysaccharidoses? | Hurler syndrome and Hunter syndrome |
| What are the clinical findings of Hurler syndrome? | Developmental delay, gargoylism, airway obstruction, corneal clouding, hepatosplenomegaly. |
| Deficiency of alpha-L-iduronidase. Dx? | Hurler syndrome |
| What is accumulated in Hurler syndrome and Hunter syndrome? | Heparan sulfate, dermatan sulfate |
| What is the mode of inheritance of Hunter syndrome? | X-linked recessive |
| Mild Hurler + aggressive behavior, no corneal clouding. Dx? | Hunter syndrome |
| Which enzyme is deficient in Hunter syndrome? | Iduronate-2-sulfatase |
| What are classical findings of Tay-Sachs disease? | Progressive neurodegeneration, developmental delay, "cherry-red" spot on macula, lysosomes with onion skin, no hepatomegaly. |
| Progressive neurodegeneration + "cherry-red" spot on macula + Lysosomes with onion skin. Dx? | Tay-Sachs disease |
| Deficiency in Hexosaminidase A. Dx? | Tay-Sachs disease |
| Accumulation of GM2 ganglioside. Dx? | Tay-Sachs disease |
| What substrate is accumulated in Tay-Sachs disease? | GM2 ganglioside |
| Which lysosomal storage disease is seen with Hepatosplenomegaly, Tay-Sachs or Niemann-Pick disease? | Niemann-Pick disease |
| Which are the two Sphingolipidoses seen with "cherry-red" spot on macula? | Tay-Sachs disease and Niemann-Pick disease |
| Which is the only Sphingolipidoses with XR inheritance? | Fabry disease |
| Histology of Tay-Sachs disease? | Lysosomes with onion skin |
| What is the early triad of Fabry disease? | 1. Episodic peripheral neuropathy 2. Angiokeratomas 3. Hypohidrosis |
| What are the late manifestations of Fabry disease? | Progressive renal failure and Cardiovascular disease |
| What enzyme is deficient in Fabry disease? | a-galactosidase A |
| What substrate is accumulated in Fabry disease? | Ceramide trihexoside |
| Increased levels of Ceramide trihexoside. Dx? | Fabry disease |
| Central and peripheral demyelination with ataxia, dementia, are clinical findings of _______________________. | Metachromatic leukodystrophy |
| Arylsulfatase A deficiency leads to development of __________________________. | Metachromatic leukodystrophy |
| Which substrate is accumulated in Metachromatic leukodystrophy? | Cerebroside sulfate |
| Which myelinating cells are destroyed in Krabbe disease? | Oligodendrocytes |
| Enzyme deficient in Krabbe disease | Galactocerebrosidase |
| Which lysosomal storage condition presents with globoid cells? | Krabbe disease |
| Elevated Galactocerebroside and psychosine are seen in: | Krabbe disease |
| What are key findings of Krabbe disease? | Peripheral neuropathy, obstruction of oligodendrocytes, developmental delay, optic atrophy, and globoid cells |
| What is the most common Lysosomal storage disease? | Gaucher disease |
| What enzyme is missing or deficient in Gaucher disease? | Glucocerebrosidase |
| What are Gaucher cells? | Lipid-laden macrophages resembling crumpled tissue paper |
| Which Lysosomal storage diseases associated with Pancytopenia? | Gaucher disease |
| Another way to refer to Glucocerebrosidase? | B-glucosidase |
| What are key bone features of Gaucher disease? | Osteoporosis, avascular necrosis of femur, and bone crisis |
| Enlarged liver and spleen, and constant bone fractures + tender femur. Dx? | Gaucher disease |
| What enzyme is deficient in Niemann-Pick disease? | Sphingomyelinase |
| The absence of Sphingomyelinase in _________________ disease, leads to accumulation of ___________________. | Niemann-Pick disease; Sphingomyelin |
| (+) Foam cells + Increased levels of Sphingomyelin. Dx? | Niemann-Pick disease |
| What condition of lysosomal storage is seen with Foam cells? | Niemann-Pick disease |
| What are the classic findings of Niemann-Pick disease? | Progressive neurodegeneration, hepatosplenomegaly, foam cells, and "cherry-red" spot on macula |
| What are foam cells? | Lipid-laden macrophages, seen in Niemann-Pick disease |
| What race is in increased risk of developing Tay-Sachs, Niemann-Pick, and Gaucher disease? | Ashkenazi Jews |
| In fatty acid synthesis what needs to be transported from the Mitochondria to the Cytosol? | Citrate |
| What tissues have the highest levels of fatty acid synthesis? | Liver, lactating mammary glands, and adipose tissue |
| Fatty acid degradation goes from the ______________ to the ____________. | Cytoplasm ---> ---> Mitochondria |
| What is the required transport in Long-chain fatty acid degradation? | Carnitine-dependent transport |
| What is the main defect in Systemic Primary (1) carnitine deficiency? | Inherited defect in transport of LCFAs ito mitochondria --> toxic accumulation |
| Hypoketotic Hypoglycemia is seen with: | Primary carnitine deficiency and Medium-chain acyl-CoA dehydrogenase deficiency |
| What is the pathogenesis of Medium-chain acyl-CoA dehydrogenase deficiency? | Decreased ability to break down fatty acids into acetyl-CoA leading to accumulation of fatty acyl carnitines in the blood with hypoketotic hypoglycemia |
| What is probable cause of death in an infant in a prolonged fasting state? | Medium-chain acyl-CoA dehydrogenase deficiency |
| Associated conditions that increase the production of Ketone bodies: | Prolonged starvation, Diabetic Ketoacidosis, and alcoholism |
| Which TCA product is either depleted or stimulated into malate, that causes an increase in Ketone production? | Oxaloacetate |
| Two commonly ketone bodies used in the muscle and brain? | Acetoacetate and B-hydroxybutyrate |
| Ketone bodies give what kind of breath smell? | Fruity odor |
| A patient with a fruity breath smell is suspected of elevated ________________. | Ketone bodies |
| Which cells cannot utilize ketones? | RBCs |
| What enzyme is used in ketone production? | HMG-CoA lyase |
| How many calories are per gram of carbohydrate and protein? | 4 calories |
| 1 gram of alcohol has how many calories? | 7 calories |
| 1 gram of fatty acid is equal to _____________ kcal. | 9 |
| What is the major source of glucose in a fasting state? | Hepatic glycogenolysis |
| What is the source of glucose in a fed state? | Glycolysis and aerobic respiration |
| In a fasting state, what stimulates the use of fuel reserves? | Glucagon and epinephrine |
| In starvation, glycogen is depleted after how many days? | 1 day |
| Why can't RBCs use ketones for energy? | Lack mitochondria |
| What are some ways blood glucose is maintained in a person in starvation for 1-3 days? | 1. Hepatic glycogenolysis 2. Adipose release of FFA 3. Muscle and liver, which shift fuel use form glucose to FFA 4. Hepatic gluconeogenesis |
| What is the main source of energy in a starvation state greater than 3 days? | Ketone bodies |
| What occurs once all adipose stores, that produce ketone bodies, are depleted? | Vital protein degradation accelerates, leading to organ failure and death |
| What is the function of Hepatic Lipase? | Degrades Triglycerides remaining in IDL |
| Degrades TGs stored in adipocytes | Hormone-sensitive lipase |
| What is the role or function of Lecithin-cholesterol acyltransferase? | Catalyzes esterification of 2/3 of plasma cholesterol |
| What is the role of Lipoprotein lipase? | Degreades TGs circulating chylomicrons and VLDLs |
| Where is Lipoprotein lipase (enzyme) found? | Vascular endothelial surface |
| What enzyme degrades dietary TGs in the small intestine? | Pancreatic lipase |
| What enzyme helps the conversion of Nascent (immature) HDL into mature HDL? | LCAT |
| What is the function of Apolipoprotein E? | Mediates remnant uptake |
| Apo E is seen in which types of cholesterol? | All |
| What form of cholesterol has Apo A-I? | HDL |
| What is the role of Apo-A-1? | Activates LCAT |
| The lack of Apo AI leads to ----> | No mature HDL |
| Lipoprotein lipase Cofactor that Catalyzes Cleavage. | Apo C-II |
| Which cholesterol presentations have Apo C-II? | Chylomicron, VLDL, adn HDL |
| What is the function of Apo B-48? | Mediates chylomicron secretion into lymphatics |
| Apo B-48 is positive for: | Chylomicrons and Chylomicron remnant |
| Function of Apo B-100? | Binds LDL receptor |
| Only particles form the ______________ have Apo B-100 activity. | Liver |
| What are lipoproteins made of? | Cholesterol, TGs, and phospholipids |
| Which type of Lipoproteins carry the highest amount of cholesterol? | LDL and HDL |
| LDL transports cholesterol from: | Liver to tissues |
| HDL transports cholesterol from: | Periphery to Liver |
| Which is the "healthy" cholesterol? | HDL |
| Why is HDL "healthy" cholesterol? | Brings peripheral cholesterol to the liver to be metabolised and excreted. |
| What is the function and role of cholesterol? | Needed to maintain cell membrane integrity and synthesize bile acid, steroids, and vitamin D |
| Which cells secrete chylomicrons? | Intestinal epithelial cells |
| What are the delivery paths of Chylomicrons? | 1. Delivery TGs to peripheral tissues 2. Delivers cholesterol to liver in the form of chylomicron remnants |
| Delivers hepatic TGs to peripheral tissue. | VLDL |
| What is the delivery done by LDL? | Hepatic cholesterol to peripheral tissues |
| How is LDL formed? | By hepatic lipase modification of IDL in the liver and peripheral tissue |
| How is LDL taken up by target cells? | Via receptor -mediated endocytosis |
| What is the main function of HDL? | Mediates reverse cholesterol transport from periphery to liver |
| Repository for apolipoproteins C and E? | HDL |
| Alcohol increases the synthesis of which lipoprotein? | HDL |
| What organs usually secrete HDL? | Liver and intestine |
| Which lipoproteins are absent in Abetalipoproteinemia? | Chylomicrons, VLDL, and LDL |
| Which Apolipoproteins are absent in Abetalipoproteinemia? | ApoB-48 and ApoB-100 |
| What are the late manifestations of Abetalipoproteinemia? | Retinitis pigmentosa, spinocerebellar degeneration due to vitamin E deficiency, progressive ataxia, acanthosis |
| What is the treatment for Abetalipoproteinemia? | Restriction of Long-Chain fatty acids and large doses of Vitamin E |
| Reason for spinocerebellar degeneration in Abetalipoproteinemia? | Vitamin E deficiency |
| Type I dyslipidemia is known as: | Hyperchylomicronemia |
| What are the clinical manifestations of Hyperchylomicronemia? | Pancreatitis, hepatosplenomegaly, and eruptive/pruritic xanthomas; Creamy layer in supernatant |
| What is the pathogenesis of Hyperchylomicronemia? | Lipoprotein lipase or apolipoprotein C-II deficiency |
| What is lipids are elevated in Type I familial dyslipidemia? | Chylomicrons, TG, and cholesterol |
| Another name for Type II Familial Dyslipidemia? | Familial hypercholesterolemia |
| What is the pathogenesis of Familial hypercholesterolemia? | Abscent of defective LDL receptors, or defective ApoB-100 |
| What are the clinical manifestations of Familial hypercholesterolemia? | Accelerated atherosclerosis, tendon xanthomas, and corneal arcus |
| What is the most commonly affected tendon by a xanthoma in Familial hypercholesterolemia? | Achilles |
| Defective LDL receptor. Dx? | Familial hypercholesterolemia |
| Defective ApoB-100. Dx? | Familial hypercholesterolemia |
| Defective ApoE. Dx? | Dysbetalipoproteinemia |
| In type IIa Familial hypercholesterolaemia, what are the main blood lipids elevations? | LDL and cholesterol |
| Besides LDL and cholesterol, what other lipid is increased in blood in Type IIb Familial hypercholesterolemia? | VLDL |
| What are the clinical manifestations of Dysbetalipoproteinemia? | Premature atherosclerosis, tuberoeruptive xanthomas, palmar xanthomas |
| Hepatic overproduction of VLDL, describes the pathogenesis of which familiar dyslipidemia? | Hypertriglyceridemia |
| What is the common name of Type IV familial dyslipidemia? | Hypertriglyceridemia |
| What another name for Type III familial dyslipidemia? | Dysbetalipoproteinemia |
| Which are the Autosomal recessive Familial dyslipidemias? | Type I and Type III |
| Type II and Type IV familiar dyslipidemias are of __________ __________ inheritance. | Autosomal Dominant |
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rakomi
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