Mixed open Q Flashcards
Elevation of orotic acid
little pyrimidine synthesis, high breakdown
AMP deaminase deficiency
dATP ↑ DNA ↓ methylation ↓ cAMP ↑ and causes immune deficiency
Free ring in nucleotide synthesis
pyramidene has free ring (orotic acid) and PRPP is added
purine does not have free ring
Activation of star
ACTH
Hyperuricemia is caused by
McArdles disease Fructose intolerance G6 phosphatase ↓ (von Gierke) HGPRT ↓ Chemotherapy
Common in alpha keto glutamate dehydrogenase and branched amino acid dehydrogenase and pyruvate dehydrogenase
Same E3 component in enzyme complex
Inactive glycogen phosphorylase
E.G: mc addle
F1p ↑↑
Pi↓
uses inorganic phosphate, but no glycogenolysis will happen
ATP level decreases and gluconeogenesis does not happen either
hypoglycemia will happen
Hypoglycemia
inactive glycogen phosphorylase
Rate limiting reaction of cholesterol synthesis
HMGCoA reductase
Substrate and product of rate limiting reaction of cholesterol synthesis
HMGCoA -> Mevalonate
Regulation of HMGCoA reductase
Allosteric regulation: cholesterol inhibits (negative feedback)
Degradation: lane sterol causes polyubiqination
Induction of enzyme:
INSIG makes no cholesterol be synthesised
LCAT reaction
phosphatidylcholine + cholesterol becomes lysolectinin + cholesterolesther
occurs in blood plasma (HDL)
usually ACAT
Synthesis of primary bile acid
conjugated and hydroxylated in the liver
hydroxyl on C3 and C7
NADH is formed
Synthesis of secondary bile acids
deconjugated and dehydroxylated at C7 in the intestine
Lesh-Nyan syndrome
deficient HGPRT (hydroxithian glutamate phosphoriboxyl transferase)
retardation
gout
self damage
aggression
little GMP and IMP
Trifunctional CAD1 enzyme
CPS2, aspartate carbonyl transferase and dihydroorotase
Dihydroorotate dehydrogenase
in mitochondria
UMP synthase bifunctional enzyme CAD2
contains orotate phosphoribzosyltransferase
OMP decarboxylase
Orotic acid acidemia
deficiency in UMP synthase
CPS2 reaction
CO2 + Gln +H20 ↔ carbamoyl phosphate + 2ADP +Pi + Glu
AlphaKB dehydrogenase reaction
alphaKB + NAD + CoA-SH ↔ propynyl coA + NADH + H
coenzymes for CYP7A1
vitamin C
NADPH
NAD
CoA
Aspirin
irreversible acetylation of COX1/2
decrease in TXA2 and PGI2
no platelet aggregation
Desaturation of fatty acids
no ATP is used
Synthesis of 1 cholesterol molecule
18 acetylcoa
36 ATP
20 NADPH
21 enzymes
PCSK9
binds to LDL receptor to cause their lysosomal degradation
LXR + oxysterol
more bile acid synthesis (increase in CYP7A1)
more cholesterol efflux (increase in ABCA1)
less LDL receptor (more IDOL)
FXR + bile acids
more bile acid resorption
more bile acid efflux
more LDL receptor (less PCSK9)
Fatty acid oxidation coenzymes
FAD, NAD, CoA
B-oxidation enzymes by order
Acyl CoA DH ( FAD)
Enoyl Hydratase
Hydroxyacyl DH
Thiolase
(O-HOT)
Rate limiting step in fatty acid synthesis
ACC (using CoA)
need ATP and bicarbonate and biotin
FA synthase complex
Domain 1:
Acetyl ACP transferase + Malonyl ACP transferase becomes condensation enzyme
Domain 2:
Ketoacyl ACP reductase becomes betahydroxylacyl ACP dehydrates and then ENoyl app reductase
Ketone bodies
acetone, acetoacetate, betahydroxybyturate
Ketogenesis
Thiolase
HMGCoA synthase
HMGCoA lyase
Acatoacetate decarboxylase or beta hydroxybutyrate DH
PP1 (protein phosphatase 1) regulation
active: insulin
inhibit: glucagon
PP1 action
dephosphorylates and activates the glycogen synthase
dephosphorylate and inhibit glycogen phosphorylase kinase and glycogen phosphorylase
Enzymes that incorporates free ammonia to organic compounds
glutamine synthase
carbamoyl phosphate I
glutamate dehydrogenase
Serine-hydroxymethyltransferase reaction
serine + THF = Glycine + H2O + THF-methyl