Topnotch - Genesis Flashcards
Components of nucleotide
phosphate group
nitrogenous base
sugar
Drug Induced Lupus
methyldopa sulfa drugs hydralazine isoniazid procainamide phenytoin etanercept
Histones are rich in
lysine
leucine
Rate limiting enzyme of de novo purine synthesis
glutamine PRPP amidotransferase
Can cause SCID
adenosine deaminase (ADA) deficieny
Metabolizes Azathioprine and 6 MP
Xanthine oxidase
Rate limiting enzyme in PYRIMIDINE synthesis
CPS II
Orotic aciduria treatment
Uridine
Lactose
galactose + glucose
Sucrose
fructose + glucose
Sites of Na dependent entry of glucose
intestinal epithelium
renal tubules
GLUT 1
brain
RBC
GLUT 4
fat tissue
skeletal muscle
Rate limiting enzyme GLYCOLYSIS
PFK1
PFK 1 (-)
citrate
ATP
PFK1 inducers
AMP
fructose 2,6 bisphophate
Activates pyruvate kinase
fructose 1,6 bisphosphate
(-) pyruvate kinase
ATP
alanine
Form of cholesterol in chylomicrons
cholesteryl ester
Cells that synthesizes chylomicrons
enterocytes
Packaged inside chylomicrons
TGL
cholesteryl esters
vit ADEK
Synthesize HMG-CoA
HMG-CoA synthase
Enzyme in cholesterol synthesis inhibited by statin
HMG-CoA reductase
Lipoproteins that deliver CHOLESTEROL and TGL to the body
VLDL
HDL
Apoproteins on HDL
apo I
apo C-II
apo E
Apoproteins on HDL that are donated to other lipoproteins
apo C-II
apo E
LCAT function
esterifies cholesterol
Apoproteins on VLDL
apo B100
apo CII
apo E
Remove TGL from VLDL
lipoprotein lipase
CETP
Remove TGL from IDL
hepatic lipase
CETP
HDL is a repository for which apolipoprotein
apo C-II
apo E
Must be present for GKRP to inactivate glucokinase
F6P
Enzyme that commits glucose to glycolysis
PFK1
Inhibited by alanine in glycolysis
pyruvate kinase
Most affected by pyruvate kinase deficiency
RBC
Intermediate in the conversion of pyruvate to PEP in gluconeogenesis
OAA
Converts pyruvate to OAA in gluconeogenesis
pyruvate carboxylase
Converts OAA to PEP in gluconeogenesis
PEP carboxykinase
Cofactor of pyruvate carboxylase
ATP
Biotin
CO2
Cofactor required by PEP carboxykinase
GTP
Biotin is cofactor for which enzymes
pyruvate carboxylase
acetyl CoA carboxylase
propionyl CoA carboxylase
Rate limiting for gluconeogenesis
FBPase I
Location of glucose 6 phosphatase
liver
kidney
Organelle where glucose 6 phosphatase is active
ER
Glycogen is most abundant
muscle
liver
Lysosomal enzyme that converts glycogen to glucose
alpha 1,4 glucosidase
Produced by glycogen phosphorylase
G1P
Enzymes that glucagon phosphorylates
glycogen phosphorylase
glycogen synthase
Von Gierke Treatment
frequent cornstarch ingestion b/w meals
avoidance of fructose and galactose
MC cause of death in Pompe disease
HF (hypertrophic cardiomyopathy)
Glycogen storage disease presents in adolescence or early adulthood
myoglobinuria AFTER exercise
Mc Ardle disease
Genetic inheritance of Glycogen storage diseases I - IV
autosomal recessive
Accumulation of limit dextrins in cytosol
Cori disease - debranching enzyme deficiency
Converts G6P to phosphogluconolactone
G6PD
Storage molecule produced through the action of G6PD
NADPH
Produced at the end of the oxidative portion of HMP shunt
ribulose 5 phosphate
2 NADPH
CO2
G6PD deficiency
x-linked recessive
PBS finding in G6PD deficiency
Heinz bodies (bite cells)
Anti malarial that can precipitate G6PD deficiency
Primaquine
Quinidine
Location of HMP shunt
cytosol
Enzyme in HMP shunt that requires thiamine as cofactor
transketolase
Enzyme in respiratory burst that requires NADPH as cofactor
NADPH oxidase
Rate limiting enzyme in the oxidative part of HMP shunt
G6PD
Carbohydrates taken up into the enterocytes by SGLT1 transporter
glucose
galactose
Clinical presentation of essential fructosuria
fructose in blood and urine
Cofactors for pyruvate dehydrogenase complex
thiamine lipoic acid CoA FAD NAD
Symptoms of Dry Beriberi
polyneuropathy
symmetrical muscle wasting
Symptoms of Wet Beriberi
tachycardia
high output HF
edema
Poison that (-) lipoic acid
arsenic
PDH complex deficiency
x-linked recessive
Purely ketogenic AA
leucine
lysine
Disorders of thiamine deficiency
Beriberi
Wernicke-Korsakoff syndrome