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42 Cards in this Set

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  • Back
What is the process of metabolizing alcohol?
ethanol -> acetaldehyde -> acetate
What part of the metabolism of alcohol creates so many problems?
Alcohol forces NAD+ toward NADH
What is the Cori Cycle?
It is the recycling of lactate to pyruvate
Pyruvate (M.) -> Lactate (M.) -> Lactate (B.) -> Lactate (H.) -> Pyruvate (H.) -> Pyruvate (B.) -> Pyruvate (M.)
What determines the capacity to metabolize EtOH?
The ability to shuttle NADH into the mitochondria, which after some point becomes saturated
What happens after the NADH shuttling is saturated?
It is no longer oxidized to NAD+, depleting levels of NAD+, reducing glycolytic flux at glyceraldehyde-3-phosphate, which stops the ability to produce energy in glycolysis
What effect does alcohol metabolism have on gluconeogenesis?
Alcohol metabolism inhibits gluconeogenesis, because it drives the equilibrium between pyruvate and lactate toward lactate, thus inhibiting conversion to glucose
Where does all ethanol metabolism occur?
Liver tissue
Where does all gluconeogenesis occur?
Liver tissue
How does alcohol metabolism affect adipose tissue?
High NADH alters fat oxidation by altering the reactions for the glycerol phosphate shuttle
What happens to the acetate produced from alcohol metabolism?
converted to acetyl-CoA, but since TCA is slowed, it cannot be oxidized and must be converted to fat in the liver
What effect does the inhibited glycerol-3-phosphate dehydrogenase have on fats?
leads to more glycerol-3-phophate, the backbone for triglycerides
this leads to hyperlipidemia
What causes the hangover effects?
the acetaldehyde forms adducts with proteins and nucleic acids leading to acute toxic effects
What is cirrhosis?
It is the death of hepatocytes, caused by too much fat in the liver
cirrhosis causes the cells to become hard
Where is fructose metabolized?
primarily in the liver, kidney and small intestine
What enzyme in the liver converts fructose to F1P?
Fructokinase
What enzyme phosphorylates fructose in tissues other than liver?
hexokinase
What does lactose consist of?
a dimer of glucose and galactose
What does sucrose consist of?
a dimer of glucose and fructose
What is another name for fructose-1-phosphate aldolase?
Aldolase B
What causes "Essential fructosuria?"
deficiency of fructokinase in liver
What causes "hereditary fructose intolerance?"
deficiency of fructose-1-phosphate aldolase in liver, kidney cortex, and small intestine
What causes "hereditary fructose-1,6-BPase deficiency?"
deficiency of fructose-1,6-bisphosphatase in liver
What are the symptoms of essential fructosuria?
hyperfructosemia
fructosuria
What is hyperfructosemia?
Too much fructose in the blood
What is fructosuria?
Fructose in the urine
What are the symptoms of hereditary fructose intolerance?
Severe hypoglycemia
Vomiting on intake of fructose
Infants:
Poor feeding
Vomiting
Hepatomegaly
Jaundice
Proximal Renal Tubular Syndrome
What would happen to a patient with hereditary fructose intolerance if they failed to restrict fructose in their diet?
It would lead to liver failure and death
What is the effect of hereditary fructose intolerance?
Can't convert F1P to glyceraldehyde and DHAP
What is the effect of essential fructosuria?
can't phosphorylate fructose in cell
What is the effect of F-1,6-BPase deficiency?
Can't convert lactate to pyruvate
What are the symptoms of F-1,6-BPase deficiency?
Episodic hyperventilation due to lactic acidosis
Hypoglycemia
Apnea
Ketosis
What is lactic acidosis?
A build-up of lactic acid in the tissues and blood, which lowers physiological pH
Why are neonates with F-1,6-BPase deficiency more likely to show severe symptoms?
Their ability to perform gluconeogenesis is impaired
Where is galactose metabolism most prevalent?
Liver tissue
What does GALK stand for?
Galactose Kinase
What does GALT stand for?
Galactose uridylyltransferase
What does GALE stand for?
Galactose epimerase
What are the symptoms of classic galactosemia due to loss of GALT?
Poor feeding, weight loss, vomiting, diarrhea, lethargy
Infants: Jaundice, hepatomegaly
What is a main cause of bleeding disorders?
Liver dysfunction
What are the symptoms of GALK deficiency?
cataracts and symptoms like GALT deficiency, but not as severe
Where is the majority of GALE deficiency presented?
erythrocytes
Why is the most common form of GALE deficiency not very severe?
erythrocyte turnover allows faulty cells to be discarded