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52 Cards in this Set
- Front
- Back
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When breaking down ehtanol, what is the final product and what is the middle product?
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Ethanol-> Acetaldehyde via alcohol dehydrogenase
Acetaldelehyde-> Acetate via acetaldehyde dehydrogenase Both require NAD+ |
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A patient comes with methanol or ethylene glycol poisoning, what would you give them?
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Fomepizole, inhibits alcohol dehydrogenase
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Your friend gets drunk and you want to teach them a lesson and give them a really bad hangover, what could you give them beside cheap tequila?
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Disulfiram (Antabuse)- inhibits acetaldehyde dehydrogenase leaving a build up of acetaldehyde (thought to cause hangover symptoms)
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Would you expect to hyopglycemia in alcholics?
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Why yes, due to increase in NADH/NAD+ ratio in the liver.
Pyruvate to lactate and oxaloacetate to malate Inhibits gluconeogenesis |
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Your watching a television commercial about starving children in Africa as you eat your big mac. You think to yourself, that kids got...
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Kwashiorkor malnutrition- protein deficient diet
edema, liver fatty changes, anemia, skin lessions |
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Marasmus malnutrition can be summarized as
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muscle wasting due to malnutrition
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Pg. 101-102
Summary of Pathways |
HAHAHA
Good luck with that shit |
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Whats the difference between Hexokinase and glucokinase?
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Well for starters both add phosphate to glucose to start glycolysis
Hexokinase is found everywhere and is inhibited by its product Glucokinase is in the liver and pancreas. High Vmax, helps store excess glucose after a meal in the liver |
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Whats the difference between fed and fasted state in terms of cAMP
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Fasted- increase glucagon, increase cAMP, less glycolysis
Fed- increase insulin, decrease cAMP, more glycolysis |
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How does Arsenic work in the body?
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Inhibits lipoic acid which needed in order to convert pyruvate into acetyl CoA
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Pyruvate Dehydrogenase deficiency
(PD converts Pyruvate to Acetyl CoA) |
Causes back up resulting in increase in lactic acid.
Will see neuro defects TX: higher intake of lysine and leucine in high fat content |
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Citrate Is Krebs Starting Substrate For Making Oxaloacetate
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Cheap way to remember all TCA steps
Citrate Isocitrate alpha Ketoglutarate Succinyl CoA Succinate Fumarate Malate Oxaloacetate |
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What does the TCA cycle produce
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3 NADH
1 FADH2 2 CO2 1 GTP in total 12 ATP/ acetyl CoA times 2 for glucose |
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What gets you more ATP
NADH or FADH2 |
NADH gets you 3 ATP compared to only 2 from FADH2
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HMP shunt pathway is converts what to what?
And why is it useful? |
glucose-6-phosphate to ribose and NADPH to be used for fatty acid or steroid synthesis
G6P dehydrogenase is the oxidative enzyme and is not reversible Transketolase is the nonoxidative enzyme and is reversible |
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Chronic Granulomatous Disease is what?
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When you have a NADPH oxidase deficiency so you can't convert o2 to o2 radical in a neutrophil or monocyte
impaired immune response |
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What do you need Glucose-6-phosphate dehydrogenase for and what happens if you have a deficiency?
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In red blood cell a lack G6P dehydrogenase causes a decrease in NADPH which in return leads to increase hemolytic anemia because NADPH protects against free radicals.
Get Heinz bodies- oxidized hemoglobin in RBC |
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What is a bite cell?
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Whats left after a macrophage takes a bite out of an RBC to remove a Heinz body.
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Essential Fructosuria
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You have a defect in fructokinase and you find fructose in the blood and urine
it is benign and asymptomatic |
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What major defect is found with Fructose Interloerance
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a deficiency in Aldolase B
Can't break down fuctose-1-P This causes a decrease in available phosphate hypoglycemia, jaundice, cirrhosis, vomiting Eat less fructose damit |
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Galactokinase deficiency is a mild or serious condition?
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Mild, galactose appears in blood and urine. Might see some infantile signs (late smile, cant track object)
Autosomal recessive |
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Absences of glactose-1-P uridyltransferase leads to what?
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Classic galactosemia
Accumlation of toxic substances causes faiulure to thrive, jaundice, mental retardation Tx- exclude galactose and lactose from diet |
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How can the body convert glucose to fructose in order to trap glucose in a cell?
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glucose to sorbitol via aldose reductase
sorbitol to fructose via sorbitol dehydrogenase |
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Tissues lacking the ability to convert sorbitol to fructose are in danger of what?
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Sorbitol accumulation
osmotic damage such as cataracts, retinopathy, neuropathy |
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What two amino acids are required during growth?
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ARG and HIS
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How is ammonium transported out of the muscle and into the liver?
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Alanine and glutamate are used to to transport ammonium.
Called the Cori cycle |
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How does Hyperammonemia inhibit the TCA cycle?
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Causes excess NH4+ to use up all the alpha-ketoglutarate causing the TCA cycle to stop
S&S- tremor, slurring of speech, blurry vision |
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What is the most common urea cycle disorder?
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Ornithine transcarbamoylase deficiency
X-linked recessive. Will find orotic acid in blood and urine |
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What vitamins are need to convert dopa to dopamine then dopamine to NE?
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B6 converts dopa to dopamine
Vit C converts dopamine to Norepi |
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If you find a baby to have phenylketonuria, you think to yourself, no fake sugar for you.
Why? |
Defect in converting phenylanine into tyrosine.
Want to decrease phenylanine and increase tyrosine intake Findings with phenylkeotonuria is mental retardation, growth retardation, seizures, musty body oder |
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Alkaptonuria?
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congenital defect of homogentisic acid oxidase.
Can't break down tyrosine into fumarate Mostly benign, will see urine turn dark with exposure to air |
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How do ablinos become so day walker like.
(day walker-> vampire-> dont see the sun-> very white) |
Defect in trosinase which is autosomal recessive. Cant synthesis melanin
Defect in tryosine transporters less tyrosine so less melanin |
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There are 3 causes of homocystinuria (true story)
How would you treat them all |
more B12 to convert HC to Methionine
more B6 to convert HC to cystathinonine cystathionine synthase deficiency ( need more b12 and folate and Cys) |
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You find cystine kidney stones in a patient.. whats up?
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think renal tubular amino acid transporter defect.
Tx- alkalinize the urine with acetazolamide |
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Maple syrup urine disease
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cant break down branched amino acids
Ile, Leu, Val ( I Love Vermont) issue is with decrease alpha ketoacid dehydrogenase |
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Hartnup Disease
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defect in neutral amino acid transporter on renal and intestinal epithelial cells.
Will see tyrptophan excretion in the urine. Causes Pellagra (B3 deficiency) |
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How does insulin deactivate Glycogen phosphorylase kinase?
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With protein phosphatase which removes a phosphate
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how does the muscle let itself know hey were moving and we need glycogenolysis for some energy please
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ca++ and calmodulin activate phosphorylase kinase
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You find a patient with severe fasting hypoglycemia, lots of glycogen in their liver and hepatomegaly.
you suspect a glycogen storage disease, which one? |
Von Gieke's disease which you have a deficiency in glucose6-phosphatase
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What enzyme is deficient in Pompe's disease?
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glycogen storage disease in which Lysosomal alpha 1,4-glucosidase doesn't work
Cardiomegaly which eventually leads to early death |
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Cori's disease is a milder form of Von Gierke's disease but has what enzyme deficiency?
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issue with debranching enzyme
gluconeogenesis is intact though |
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What glycogen storage disease causes an inability to break down glycogen in muscle?
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McArdle's disease
Issue with skeletal muscle glycogen phosphorylase Will have painful muscle cramps and myoglobinuria with exercise |
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You find a patient has an extra amount of Ceramide trihexoside and you determine that they have a lysomal storage disease, which one?
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Fabry's disease
X linked recessive!!!!! deficiency with alpha galactosidase A, will see peri neuropathy with cardiac/renal problems |
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What is the most commong lysosomal storage disease in which you see macrophages that look like crumpled tissue paper?
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Gaucher's disease!
issue with breaking down glucocerebroside S&S heptaosplenomegaly, necrosis of femur |
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Looking at a path slide, you notice a cherry spot on a macula foam cell with enlarged liver and spleen.
What disease is this? |
Niemann-Pick disease
Lysomal storage disease where you accumulate sphingomyelin |
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Tay-Sachs disease
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cherry spots on macula, lysosomes with onion skin.
lysomal storage disease, defect in hexosaminidase A leads to GM2 ganglioside build up. |
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How do you tell the difference between Tay-Sachs disease and Niemann-Pick disease since both have cherry red spots on macula?
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NP has heptaosplenomegaly while TS does not...
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Krabbe's disease questionmark
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lysosomal storage disease
S&S development delay with optic atrophy Accumulation of Galactocerebroside |
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Metachromatic Leukodystrophy
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lysosomal stroage disease with central and peripheral demyelination
ataxia follows. Cerebroside sulfate build up due to arylsulfatase A not working |
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Hurler's syndrome is awesome because?
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Gargoylism (yep its what it sounds like)
lysosomal storage disease, due to build up of sulfates corneal clouding and hepatosplenomegaly are common |
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Hunter's syndrome is a lysosomal storage disease that resembles what other disease?
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Hurler's but milder and X link recessive
no corneal clouding but will have aggressive behavior (theoretically you could have an aggressive gargoyle) |
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All lysosomal storage diseases are autosomal recessive except which two?
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Hunter's and Farby's
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