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116 Cards in this Set
- Front
- Back
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Alanine?
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A, ala
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Cysteine?
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C, cys
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Aspartic acid?
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D, asp
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Glutamic acid?
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E, glu
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Phenylalanine?
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F, phe
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Glycine?
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G, gly
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Histidine?
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H, his
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Isoleucine?
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I, Ile
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Lysine?
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k, lys
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Leucine?
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L, leu
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Methionine?
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M, met
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Asparagine?
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N, asn
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Proline?
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P, pro
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Glutamine?
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Q, gln
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Serine?
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S, ser
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Threonine?
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T, thr
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Valine?
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V, val
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Tryptophan?
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W, trp
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Tyrosine?
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Y, tyr
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Only ketogenic aa? 2
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Lys, leu
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Essential aa? Mnemonic?
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PVT TIM HALL: phe, val, thr, trp, ile, met, his, ala, leu, lys
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Glucogenic aa? 2 Via? 1
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Val, ile via propionyl coA
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Ketogenic aa? 3 Via? 2
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Leu, ile, lys via acetyl coA, acac
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Source?
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Sk muscle
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Steps? 4 via?
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Transamination -> oxidative decarboxylation via α-keto acid dehydrogenase -> Val & ile -> -> propionyl coA; leu -> -> Acac
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Cofactors req? 5
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Thiamine, lipoate, CoA, FAD, NAD
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MSUD: defective enzyme?
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α-keto acid dehydrogenase
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MSUD Sx 4
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Present w/ 1st meal like inf w/o fever; characteristic odor; convulsion, poor mental development
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MSUD Tx
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Diet low in BCAA but need essentials
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Modified Cori cycle? 3
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1. AT (aminotransferase) pass N from BCAA -> glu -> ALT pass N from glu -> pyr, making ala in the process -> Ala -> liver -> liver ALT convert to glu + pyr
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How differ from RBC Cori cycle?
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RBC: need LDH to generate NAD+
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Interconversion b/t ser & gly via?
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Serine hydroxymethyl transferase
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Purpose?
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Builds up FH4
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Net synth?
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1 ser/2 gly via gly cleavage
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Porphyrin ring uses?
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glycine
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Oxalate made from? Risk for?
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Glycine; kidney stones/failure
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Cr made from? 4
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gly + arg + met + methyl from SAM
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Creatine v. creatinine
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Creatine contain P, creatinine doesn't
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Normal level?
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1 mg/dL
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Used for checking? 3
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Kidney fxn, urine strength, muscle wasting
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Kinetics? Limiting reagent?
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0-order kinetics; NAD+
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Fx on nervous sys? 3
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Depressant that incr GABA-mediated inhibition, Inh Ca thru volted channels, Inh NMDA-R
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EtOH dehydrogenase w/ highest spec?
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ADH1
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Gastric ADH inh by? M v. F?
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Inh by aspirin, H2 blockers; F > M
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Fomepizole rxn
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Inh ADH -> methanol & ethylene glycol poisoning
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Disulfuram rxn
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Aldehyde buildup by block aldehyde dehydrogenase (AlDH)
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Acetate convert to? By?
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Acetate -> acetyl CoA by acetyl coA synthase
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MEOS rxn?
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MEOS Km > ADH Km; Uses NADPH to convert EtOH -> acetaldehyde
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EtOH fx on P450?
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Induction of CYP2E1 on ER -> incr clearance
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Metabolic fx of EtOH: 6
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Inc: lactate/pyr, malate/OAA, glycerol-P/DHAP, TG; Dec: gluconeogenesis, beta-oxidation
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Induces
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Hyperlipidemia, ketoacidosis, hyperuricemia
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Acetaldehyde fx: 3
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Protein adducts -> Ab; Deplete GSH oxidative stress; Mitochondrial dysfxn
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Kcal/g?
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7 kcal/g
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Depletes which cofactors? 6
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Thiamine, pyridoxine, met, SAM, phosphatidylcholine, Vit A
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Biomarkers: 7
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ALP, ALT, AST, GGT, MCV, MAO, bili
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Glu/Gln rxn catalyzed by? 2
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Glu + ATP -> gln via gln synthetase; gln->glu + NH4+ via glutaminase
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Critical for?
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For NT regulation
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Inh by?
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Inh by NH4+
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Glu -> urea cycle?
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Glu + ATP -> -> GSA -> ornithine via ornithine aminotransferase
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Orinthine metab to?
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Ornithine -> putrescine -> sperimidines or spermines
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Glu in GSH?
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Use COOH for bond
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OAA/asp/asn rxn? 3
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OAA -> asp via PLP + transaminase; asp + gln + ATP-> asn + glu via asn synthetase; asn -> asp via asparaginase
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Arg used to make?
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NO, Cr
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GSH Made of? 3
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glu + cys + gly
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GSH Oxidized to? By?
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GSSG via glutathione peroxidase
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GSH Req what to make?
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NADPH needed to make GSH
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GSH Excretion?
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As mercapturic acid when lose gly, glu
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GSH Metab of Rx?
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Acetaminophen metab to NAPQI -> need GSH
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SAM =?
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Met w/ activated SH
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SAH =?
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S-adenosyl homocys -> breaks down to homocys
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Homocys =?
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Met w/ terminal methyl group missing
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Homocysteine -> met step 1
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Req B12-CH3 for methyl donor
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Homocysteine -> met step 2
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B12 restored by folate (cxn to ser/gly)
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Homocysteine -> met step 3
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SAM donates methyl -> becomes homocys
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Homocys -> TCA? Step 1
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homocys + ser -> cys + α-ketobutyrate via PLP (B6)
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homocys -> TCA? Step 2
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a-KB -> propionyl coA + CO2
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homocys -> TCA? Step 3
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propionyl coA + CO2 + biotin -> methylmalonyl coA
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homocys -> TCA? Step 4
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methylmalonyl coA + B12 -> succinyl coA
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Homocystinuria: definition?
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Homocys level 100 (norm 5-15)
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homocystinuria: Sx?
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Deformity of chest, lens dislocation
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homocystinuria prominent in?
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Finland, Denmark
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homocystinuria Tx?
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High B6, lower met, incr cys, folate
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Cystinuria?
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Cys = very insoluble -> gets concentrated -> stones
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norepi + SAM =
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epi
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guanidinoacetate + SAM =
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Cr
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nuc + SAM =
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methyl-nuc
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phosphotidylethanolamine + SAM =
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phosphotidylcholine
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acetyl5HT + SAM =
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melatonin
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Phe -> tyr via? req?
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Phe hydroxylase; O2 + BH4
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PKU: Sx? 2 Tx? 2 Defective enzyme?
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Phe hydroxylase defective
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Tx? 2
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MR, pigmentation def
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Defective enzyme?
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Phe-restricted diet; supply tyr
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BH4 formed from?
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BH4 from GTP
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Tyr = precurosor for? 4
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Norepi, epi, melanin, dopa
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Trp = precrusor for? 2
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5-HT, melatonin
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Excreted in?
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Hartnup dz (defective transporter)
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Predisposes to?
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Pellegra
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His glucogenic thru?
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Fi-glu (Variant of glu)
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Ala fuel for? 1
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Liver -> gluconeogenesis
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Gln fuel for? 3
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Kidney, gut, imm sys
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Val fuel for? 1
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Brain
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Transaminase rxn? 3 category
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BCAA -> α-ketoacid; ala <-> pyr; asp <-> OAA
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Gut fuels in fasting state? 3
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Gln, glucose, KB
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Gln catalyzed to? (fed state)
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Gln -> Glu -> α-KG -> TCA (makes ala -> liver)
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Gln catalyzed to? (fasting state)
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GSH -> ornithine -> citrulline -> urea cycle
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Gut fuels in fed state? 3
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Gln, glu, asp
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Intestine rxn after high protein meal? 3
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Metab gln, glu, asp for energy; Net synth ala, pyr, urea cycle intermediates
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Promoted by?
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esp w/ leu, incr insulin
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Urea cycle Mnemonic?
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Ordinarily, careless crappers are also frivolous about urination
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urea cycle Step 1
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Ornithine + carbomyl phosphate -> citrulline
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urea cycle Step 2
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Cit + asp -> argininocusccinate
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urea cycle Step 3
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arg-s -> arg + fumarate
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urea cycle Step 4
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arg -> ornithine + urea
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OTC def: genetics?
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X-linked R
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OTC def patho
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Xs carbamoyl phosphate -> orotic acid
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OTC def Sx? 3
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Orotic acid in blood/urine, dec BUN, hyperammonemia Sx
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