Term
| True or False: lipolysis and ketogenesis is boosted by insulin, which is lost in type 1 DM. |
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Definition
| false, lipolysis and ketogenesis are restrained by insulin which is lost in T1DM |
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Term
| The following labs are for what illness - Labs (blood) glucose high, HCO3- low, pH low, K+ normal or even high but body severely depleted,C-peptide low or undetectable,Insulin autoantibodies, Anti GAD antibodies positive? |
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Definition
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Term
| Which of these factors contribute to insulin resistance - central obesity, pregnancy, adolescence, sedentary lifestyle, exercise, genetics? |
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Definition
| Everything except exercise, in 80% of people the islet cell adaptive response is to increase insulin output, but in the other 20% who have T2DM insulin drops |
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Term
| True or False: increased BMI can decrease insulin demand. |
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Definition
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Term
| What is the role of metformin in T2DM treatment? |
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Definition
| Metformin improves hyperglycemia primarily through its suppression of hepatic glucose production since insulin levels tend to be low which is the normal gluconeogensis inhibitor. |
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Term
| The following nutritional supplements are necessary after what procedure - 60-100 grams of protein, Multivitamin, Calcium/Vitamin D, Iron, B-Complex,B-12? |
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Definition
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Term
| True or false: HDL-C values below 40 mg/dL are often associated with increased CHD risk even if total cholesterol is below 200 mg/dL, and hypertriglyceridemia or elevated LDL-C levels greatly enhance CHD risk in patients with low HDL-C. |
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Definition
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Term
| Which of these essential fatty acid families has more prothrombotic and inflammatory metabolites than the other - omega 6 or omega 3? |
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Definition
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Term
| What is more beneficial in lowering a person’s triglyceride levels, omega 3’ s from fish oil(EPA+DHA) or from plants (a-linoleic acid)? |
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Definition
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Term
| Which of these two facts about omega 3’s are true: 1. TG lowering: via a combination of inhibited hepatic TG synthesis/secretion and enhanced VLDL-TG clearance 2. Reduced risk for sudden cardiac death: via an increased resistance to ischemia-induced ventricular arrhythmias? |
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Definition
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Term
| What is the effect of plant-based sterols(conjugated linoleic acid), cinnamon, and viscous fiber on LDL levels? |
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Definition
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Term
| Why are some fatty acids referred to as “essential” like linoleic acid? |
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Definition
| They must be consumed in the diet |
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Term
| In the fed state, the liver takes Acetyl-CoA and metabolizes it to TAG-rich VLDL to dump into the blood, what tissue enzyme hydrolyzes that TAG-rich VLDL to release FFA for delivery to adipose tissue? |
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Definition
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Term
| In the fasted state, insulin is low, glucagons and EPI are high, so how does that release FFA’s to the blood for an energy source? |
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Definition
| The glucagons and EPI stim cAMP in fat cells, hydrolyzing TAG via the hormone sensitive lipase, and the FFA’s are transported to the liver via albumin |
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Term
| In a fasted state, like T1DM or starvation or metabolic syndrome, the liver synthesizes a large volume of ketone bodies from all the excess AcetylCoA due to TAG hydrolysis, what organelle drives the b-oxidation of these fatty acids? |
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Definition
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Term
| This organelle oxidizes very long chain fatty acids, has a role in bile acid synthesis, and metabolizes H2O2. |
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Definition
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Term
| What is the difference between glucogenic and ketogenic amino acids? |
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Definition
| Glucogenic amino acids can convert the carbon skeleton to pyruvate or a TCA cycle intermediate to form glucose; ketogenic amino acids can only form acetyl CoA to make ketone bodies and not glucose |
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Term
| Which of these diseases causes skin changes and edema - edematous protein energy malnutrition (kwashiorkor) or non-edematous protein energy malnutrition (marasmus)? |
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Definition
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Term
| True or False: PKU is an autosomal recessive disease that has a defective phenylalanine hydroxlase enzyme causing low levels of phenylalanine in the blood. |
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Definition
| False, PKU causes high levels of phenylalanine which results in mental retardation if left untreated due to the competitive inhibition of protein transport and inadequate synthesis of proteins and neurotransmitters in the brain; PKU may also be cause by BH4 deficiency. |
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Term
| What is the treatment for PKU sufferers and PKU mothers about to conceive? |
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Definition
| A strict diet in very low phenylalanine, which for newborns results in near normal intelligence and a long life. |
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Term
| What are the effects of PKU at birth, early manifestations, and in the second and third decade? |
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Definition
| Normal appearance, mental retardation, motor dysfunction |
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Term
| True or false: maple syrup urine disease is an autosomal recessive disease that results in the accumulation of branched chain keto acids that can results in metabolic acidosis and nervous system toxicity. |
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Definition
| True, and it is treated with diets low in isoleucine, valine, and leucine |
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Term
| Identify this type or diabetes: 90% occurrence in patients without family history, different prevalence in different populations, low percentages in twin concordance studies, viral environmental triggers, and locus within MHC. |
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Definition
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Term
| Identify this type of diabetes: non-insulin dependent, complex genetics due to heterogeneity, polygenic, familial clustering, twin studies discordance, greater than 90% of diabetes cases. |
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Definition
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Term
| Explain methlyation, acetylation, and imprinting. |
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Definition
| Methylation silences genes or turns them on, acetylation modifies histones to allow access to DNA, imprinting transfers the specific epigentics from both parents which can allow for improper gene expression due in part to methylation. |
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Term
| True or False: DIPC (dipalmitoyl phosphatiyl- choline) is THE major lipid of surfactant in the lungs. |
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Definition
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Term
| Which of the following is correct about lysosomal storage diseases - lysosomal enzyme deficiencies are present in all cells and tissues, genetically heterogenous, enriched in some ethnic groups (Ash Jews), all are inherited in medelian, autosomal recessive fashion except a few x-linked, usually chronic and progressive. |
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Definition
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Term
| In I-Cell disease there is a deficiency of the PG1cNAc transferase enzyme in the golgi apparatus, so what is the result? |
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Definition
| The enzymes that should be tagged to be delivered to the lysosome are dumped in the blood stream, therefore the cell components that need to be degraded won’t be and accumulate to toxic levels. |
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Term
| Defects causing the accumulation of ganglioside GM2 in Tay-Sachs disease impairs brain function, what is the result of the defect of one enzyme in the degradation of gangliosides? |
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Definition
| There is a toxic accumulation inside the cell of the unmetabolized products. Which is especially toxic to newborn neurons. |
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Term
| Which of the following are possible treatments for lysosomal storage diseases - removal of stored products(cysteamine for cystinosis export defect), substrate reduction for glycosphingolipid diseases, or providing the missing enzymes via enzyme replacement or gene therapy? |
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Definition
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Term
| List the purine and pyrimidine bases. |
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Definition
| Purines - adenine, guanine; Pyrimidine - cytosine, uracil, thymine |
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Term
| Which of the following are the cellular roles of nucleotides for each category: energy metabolism - ATP, UDP-glucose; physiological regulation - cAMP/gAMP, adenosine controls coronary blood flow; coenzyme components - FAD/NAD, CoA? |
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Definition
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Term
| De novo biosynthesis of nucleotides occurs in most cells but which organ is the major site of creation? |
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Definition
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Term
| True or False: PRPP is used for both purine and pyrimidine syntheses. |
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Definition
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Term
| Inosine monophosphate is the first nucleotide in the cycle to create purines, but it forms what important 2 signal molecules? |
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Definition
| AMP and GMP, if these then react with ribonucleotide reductase, RNR, they become dAMP and dGMP; AMP and GMP are the base for all other purine nucleotides |
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Term
| When purines (eg AMP/GMP) are degraded what is the final end product excreted as? |
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Definition
| Xanthine which is converted to Uric acid |
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Term
| Since increased uric acid production could lead to urate crystals precipitating in joints causing gout, what enzymes can you inhibit to reduce the crystal production? |
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Definition
| Allopurinol will inhibit xanthine oxidase which will stop uric acid from forming, you can also use colchicines to reduce metabolic activity of inflammatory cells |
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Term
| True or False: SCID (severe combined immunodeficiency) is a result of a deficiency of ADA, adenosine deaminase, which prevents cells from breaking down dAMP to uric acid; these high levels of dAMP inhibit the synthesis of nucleotides by blocking RNR so it becomes toxic to T and B cells. |
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Definition
| True; the only treatment of which is bone marrow transplant or gene therapy |
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Term
| In the purine salvage pathways the enzyme HGPRT can be defective causing Lesch-Nyhan Syndrome which leads to increased purine degradation, what are the symptoms? |
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Definition
| Increasing uric acid, neurological defects, and behavior problems. |
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Term
| Compare and contrast purines and pyrimidines in regards to their synthesis with ribose, name their first nucleotide, what they can be degraded to, and whether they are salvaged through their base or nucleoside. |
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Definition
| Purines are synthesized on ribose, first nucleo is IMP, degrades to uric acid, salvaged at base level; Pyrimidines are synthesized then added to ribose, first nucleotide is UMP, degraded to completely soluble structures aa/CO2/NH3, salvaged thru nucleoside |
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Term
| True or False: purines and pyrimidines have similar degradation and salvage pathways. |
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Definition
| False, similar salvage not degradation |
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Term
| How does sulfonamide inhibit folic acid synthesis in bacteria if it is an analogue to folic acids precursor? |
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Definition
| It competitively binds for the precursor’s enzyme so that the bacteria cannot make folic acid which is necessary for purine and pyrimidine synthesis. |
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Term
| Why is it that anti-cancer (6-MP,araC) and anti-viral (AZT,Acyclovir) drugs use analogues of pyrimidines and purines? |
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Definition
| Because these specific compounds affect nucleic acid synthesis and can be activated by viral enzyme in cell only or cancer cells become more suceptible because they are rapidly dividing. |
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Term
| When a person has gastric bypass surgery and develops thaimine (B1) deficiency called Berberi, what is the result symptomatically? |
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Definition
| Thiamine is absorbed primarily in the jejunum and is required for carbohydrate metabolism. Thiamine deficiency can cause peripheral neurologic, cerebral, cardiologic, and gastrointestinal manifestations. |
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Term
| adrenoleukodystrophy is a disease that results from dysfuntional peroxisomes, how does this dysfunction create severe brain damage? |
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Definition
| The peroxisomes can't import very long chain fatty acids to degrade so they build up to toxic levels in tissues |
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Term
| Alkaptonuria is an autosomal recessive disease that results in the relatively harmless accumulation and excretion of homogentisic acid, what are the main symptoms? |
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Definition
| dark urine, darkened cartilage, arthitis at old age. |
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Term
| What type of diabetes is autosomal dominant, expressed in every generation, seen in male-to-male transmission, with locus/allelic heterogeneity? |
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Definition
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