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56 Cards in this Set

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What is the first irreversible step/enzyme in gluconeogenesis? Where?
1. Pyruvate carboxylase converts Pyruvate to OAA in mitochondria
What is the next irreversible step/enzyme in gluconeogenesis? Where? what is needed?
OAA -> PEP by PEP carboxykinase in the CYTOSOL; requires GTP
What is the next irreversible step/enzyme in gluconeogenesis? Where?
F1,6-bisphosphate -> Fructose6P by F1,6Bisphosphatase; in the cytosol
What is the last irreversible step/enzyme in gluconeogenesis? Where?
Glucose-6-P -> Glucose by Glucose 6 phosphatase; in the Endoplasmic Reticulum!!
What is an acronym for the 4 enzymes required for the irreversible steps of gluconeogenesis?
Pathway Produces Fresh Glucose
-Pyruvate Carboxylase
-PEP carboxykinase
-F16Bisphosphatase
-Glucose6Phosphatase
What TISSUES contain the enzymes required for MAKING glucose via gluconeogenesis?
-LIVER
-KIDNEY
-INTESTINAL EPITHELIUM
What tissue is INCAPABLE OF making glucose?
Muscle
What will a deficiency in one of these key enzymes result in?
Hypoglycemia
What is the pathway that produces NADPH?
HMP shunt - the Pentose Phosphate Pathway
And what are 3 processes NADPH required for?
-Steroid biosynthesis
-Fatty acid biosynthesis
-GSH reductase in RBCs
WHERE does the HMP shunt occur?
ALL reactions are in the CYTOSOL
Does anything happen with ATP in the HMP shunt?
nope
What are 3 tissues where the HMP shunt is active?
-Adrenals (steroid synth)
-Liver
-Lactating breast
What are the 2 types of reactions in the HMP shunt? Which is reversible?
-oxidative - nonreversible
-nonoxidative - reversible
What is the key enzyme in the OXIDATIVE/NONREVERSIBLE step of the HMP shunt? Product?
G6P Dehydrogenase - Produces NADPH!
And what is NADPH for?
-Steroid synthesis
-Fatty acid synthesis
-Glutathione reduction
-Cytochrome P450s
What enzymes catalyze the NONoxidative, REVERSIBLE step in the HMP shunt? What are the products?
Transketolases
Produces Ribose-5-Phosphate, G3P, and F6P
What cofactor do Transketolases require?
Thiamine
What is Ribose-5-P required for?
Nucleotide synthesis
What are G3P and F6P?
Glycolytic intermediates
What is the RLS step enzyme in the HMP?
G6PD! glu-6phosphate dehydrogenase
What does G6PD deficiency cause?
Hemolytic anemia
Why does G6PD deficiency cause hemolytic anemia?
Because lack of it means GSH is not kept reduced, so free radicals build up
What are 3 oxidizing agents that will exacerbate hemolytic anemia caused by G6PD deficiency?
-Fava beans
-Sulfonamides
-Primaquine
-Tuberculosis drugs
In what race is G6PD deficiency more prevalent?
Blacks
What can be seen in RBCs that are deficient in G6PD?
Heinz bodies
What are Heinz bodies?
Hemoglobin precipitates
What is the INHERITANCE of G6PD deficiency?
X-linked recessive!
Why is G6PD deficiency more prevalent in blacks?
Confers resistance to MALARIA
What is FRUCTOSE intolerance caused by?
Hereditary deficiency of ALDOLASE B
What is the inheritance pattern of ALDOLASE B deficiency?
Recessive (its a metabolic disorder)
What accumulates in Aldolase B deficiency and what is the result?
Fructose-1-P builds up and depletes ATP availability; inhibits Glycolysis and Gluconeogenesis!
What are 4 symptoms of Fructose intolerance?
-Hypoglycemia
-Jaundice
-Cirrhosis
-Vomiting
What is the treatment for Fructose intolerance?
Decrease intake of Fructose and Sucrose!
What is Sucrose?
Glucose + Fructose
What is Essential Fructosuria?
A benign asymptomatic disorder of increased Fructose in urine due to a defect in Fructokinase
What enzyme is deficient in Galactosemia?
Galactose-1-phosphate uridyltransferase
3 Symptoms of Galactosemia:
-Mental retardation
-Hepatosplenomegaly
-Cataracts
What are the symptoms of Galactosemia caused by?
Accumulation of Galactose-1P and Galactitol which are toxic
What is the treatment for Galactosemia?
Exclude Galactose and lactose from the diet
What is lactose?
Galactose + Glucose
What will deficiency of Galactokinase cause?
Buildup of Galactose and Galactitol in serum and urine
What is LACTASE deficiency?
The hereditary or age-dependent intolerance to lactose due to loss of the brush border enzyme
3 sx of Lactase deficiency:
-Cramps
-Osmotic diarrhea
-Bloating
2 treatments for Lactase deficiency:
-Avoid lactate
-Lactase pills
What are the essential amino acids?
PVT TIM HALL
-Phe, Val, Thr
-Tryp, Ile, Met
-His, Arg, Leu/Lys
What are the ketogenic essential amino acids?
Leu and Lys
What are the Glucogenic/ketogenic AA's?
PIT
Phe, Ile, Trp
What are the Glucogenic AA's?
MATHV
Met, Arg, Thr, His, Val
What 2 AA's are essential during periods of growth?
Arg and His
What are 2 acidic AA's?
Asp and Glu
What are Aspartate and Glutamate at body pH?
Charged
What are the 3 basic AA's?
Arginine
Lysine
Histidine
Which basic aa is most basic?
Arginine
Which basic AA is not charged at body pH?
Histidine
Which aa's are high in histones? Why?
Arginine and lysine - they bind neg charged DNA.