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40 Cards in this Set
- Front
- Back
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uncouplers
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- thermogenin
- salicylate - 2,4-dinitrophenol |
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oligomycin
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- inhibits (ATP synthase)
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PFK-2
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- regualtor of glycolysis
- Active = glycolysis - inactive = gluconeogenesis - effector = F-2,6-BP upregulated by insulin inhibited by glucagon |
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Pyruvated DH
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- upregulated by INSULIN
- makes Acetyl-CoA = CAC or FA synthesis - cofactor = TPP (B1), FAD (B2); NAD (B3); lipoic acid, CoA (B5); |
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Rate limiting step of CAC
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- ISOCITRATE DH
- Isocitrate -> alpha-ketoglutarate - generate NADH Activated = ADP Inhibited = ATP; NADH |
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Dehydrogenase generate
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- NADH
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HMP shunt
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- Gluc 6 phos ----> 6- phosphogluconate ---> Ribose 5-P
- regualted by G6PDH - generated NADPH |
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NADPH
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- FA and steroid biosynthesis
- reduce glutathione which protects against ROS (GSSG -> GSH) - Respiratory burst = NADPH (O2-> O2- (superoxide)) |
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Pyruvate carboxylase
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- Pyruvate -> OAA ->> CAC or gluconeogenesis
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Galactokinase def
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- galactosemia/uria
- cataracts in childhood (ALdose reductase) tx = no galactose in diet |
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Gal-1-P uridyl transferase def
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CLASSIC GALACTOSEMIA
- galactosemia/uria - cataracts in childhood (aldose reductase) - VOMITING/DIARRHEA after milk - MR; LETHARGY; HEPATIC FAILURE tx = no galactose in diet |
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Fructokinase def
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ESSENTIAL FRUCTOSURIA
Fructosuria - does not enter cells benign |
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Aldolase B def
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FRUCTOSE INTOLERANCE
- Fructosuria - LIVER AND PROXIMAL RENAL TUBULE DISORDER Aldolase B = Fruc 1P to DHAP - tx = no fructose in diet |
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Insulin general
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- dephosphorylates enzymes
- dec blood glucose |
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Glucagon gen
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- Phosphorylates enzymes
- acts to inc blood gluc |
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Fasting state
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= 3- 36 hrs after meal
- initial inc glycogenolysis - then gluconeogenesis Muscle catabolism = supply AA for gluconeogenesis thus inc UREA Muscle = use FA Brain and RBC = glucose use remains constant |
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Starvation State
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= 3-5 days after a meal
- rely on FA adn ketones for fuel RBCs and LIVER DONOT use ketones - only enough gluconeogenesis to supply RBCs = gluconeo decreased - dec muscle catabolism to perserve muscle fxn - Ketones are markedly increased - Muscles donot use ketones = leave for brain - BRAIN = ketones primary |
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bypassing steps gluconeogenesis
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Pyruvate Carboxylase (pyr -> OAA)
PEP CARBOXYKINASE (OAA -> PEP) Fruc 1,6 -BP |
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FA synthesis
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- Acetyl CoA Carboxylase
- insulin and citrate = activate - palmitate and glucagon = decrease |
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Lipolysis
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HSL
- inc by Epi - inhibited by insulin |
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Beta oxidation
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- Carnitine Acyltransferase
- Malonlyl-CoA inhibits |
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Urea cycle
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- CPS I
- activated by N-acetylglutamate |
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Carboxylase
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- adds 1 carbon w/ help of biotin
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ketogenesis
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- HMG-CoA synthase
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Aerobic metabolism of glucose produces?
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- 32 ATP via malate-aspartate shuttle
- HEART AND LIVER - 30 ATP via glycerol-3-phosphate shuttle - Muscle |
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Anerobic glycolysis produces
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- only 2 net ATP per glucose molecule
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Glucokinase
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- only in liver and beta-cells of pancrease
- not inhibited by Gluc6_ feedback - low affinity (high Km) and high capacity (high Vmax) |
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rxns that produce ATP in glycolysis
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Phosphoglycerate kinae
- 1,3-BPG <---> 3-PG Pyruvate Kinase - PEP ----> pyruvate |
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FBPase-2 and PFK-2
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- same complex
- glucagon = inc cAMP = inc PKA = inc BPase-2 - insulin = dec cAMP = dec PKA = inc PFK2 |
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Net
glycolysis pyruvate DH |
glycolysis
2 pyruvate + 2 ATP + 2 NADH pyruvate DH acetyl-CoA + CO2 + NADH |
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Anaerobic glycolysis is the main pathway in
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RBCs
Leukocytes Kidney Medulla Lens Testes Cornea |
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TCA produces
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3 NADH + 1 FADH + 2 CO2 + 1 GTP PER ACETYL-COA
- double for glucose |
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nonoxidative portion of HMP shunt
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Transketolase = B1 required
Ribulose 5p -> Ribose 5p |
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sites of HMP shunt
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= lactating mammary glands
- liver - adrenal cortex - RBCs |
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fava beans
sulfonamides primaquine anti-TB drugs |
- oxidizing agent = HA in G6PD
- XR; Heinz; Bite cells |
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sorbitol Dehydrogenase
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- convert sorbitol to fructose
- cells lacking are at risk of osmotic damage - LENS (cataracts) - Retinopathy - peripheral neuropathy |
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Essential AA's
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PVT TIM HALL
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basic AA
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- Arg (most basic)
- Lys - His (no charge at body pH) |
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Ketogenic AA
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- Lue
- Lys |
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tx of hyperammonia
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benzoate
phenylbutyrate |
