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59 Cards in this Set
- Front
- Back
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a.a. + alpha-KG <-?-> intermediate + glutamate
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aminotransferase, B6 coenzyme
alanine <-> pyruvate aspartate <-> oxaloacetate glutamine <-> glutamate |
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glutamate <-?-> alpha-KG + NH3
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glutamate dehydrogenase
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alkaptonuria
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no homogentisate oxidase (tyr degradation)
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maple syrup urine disease
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no alpha-KG dehydrogenase
accumulation: leucine, isoleucine, valine |
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NH3 + CO2 -?-> carbomoyl phosphate
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carbomoyl phosphate synthetase I
mitochondrial matrix |
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carbomoyl phosphate syntehtase I regulation?
urea cycle |
n-acetyl-glutamate activate
arginine, protein rich meal |
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arginine -?-> urea + orinithine
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arginase
cytosol |
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glutamate + NH4 -?-> glutamine
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glutamine synthetase
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glutamine -?-> glutamate + NH4
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glutaminase
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glucose-alanine cycle
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pyruvate -> alanine -> glutamate
increase in starvation |
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hyperammonemia
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acculumation of urea cycle metabolites
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phenylalanine -?-> tyrosine + quinonoid dihydrobiopterin
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phenylalanine hydroxylase
tetrahydrobiopterin |
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inactive phenylalanine hydroxylase ->> increase phenylalanine ->> ?
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PKU
-> no tyrosine hydroxylase -> can't produce dopa, seratonin, etc |
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hyperphenylalanemia
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no dihydrofolate reductase/GTP cyclohydrolase, no tetrahydrobiopterin
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a.a. metabolic precursors of ?
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DOPA, seratonin, melanin, histamine, etc
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folic acid -?-> tetrahydrofolate (THF)
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dihydrofolate reductase
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activated methyl cycle
require THF, V-B12 |
serine + THF -> N5-methyl-THF + homocysteine -> methionine + THF
methionine -> SAM + methyl acceptor => methylated product, adenosine, honmocysteine |
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histine + THF -?-> glutamate + 5-formimino-THF
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formiminotransferase
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phosphatidylserine -?-> phosphatidylcholine
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decarboxylase, N-methyl transfersae
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B12 deficiency
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required in 1C metabolism
N5-methyl-THF accumulation |
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ribose -5-phosphate -> PRPP
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PRPP synthetase
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PRPP synthetase inhibition? activation?
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Pi activate, purine nt inhibit
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purine synthesis: PRPP -> IMP
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glutamine PRPP amidotransferase, N10-formyl-THF transfersase
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PRPP amidotransfrase inhibition? activation?
purine synthesis |
AMP/GMP/IMP inhibit, PRPP activate
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IMP -> GMP/AMP
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IMP dehydrogenase/GMP synthetase,
adenylosuccinate syntehtase/adenylosuccinase |
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purine salvage pathway
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hypoxanthine/guanine -HGPRT/PRPP-> IMP/GMP
adenine -> AMP |
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Lesche-Nyhan syndrome
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HGPRT deficiency -> all de novo purine synthesis -> hyperuricimia
excess purine bases -> uric acid -> gout |
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hypoxanthin/xanthin -?-> uric acid
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xanthine oxidase
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pyrimidine synthesis:
glutamine -?-> carbamoyl phosphate + glutamate -> OMP -> UMP |
carbamoyl phosphate synthetase II
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carbamoyl phosphate synthetase II inhibition? activation?
pyrimidine synthesis |
UTP inhibit, ATP/PRPP activate
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UTP + glutamine -?-> CTP + glutamate
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CTP synthase
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NDP -?-> dNDP
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ribonucleotide reductase
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ribonucleotide reductase inhibition? activation?
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ATP/rapid division activate, dATP inhibit
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dUMP + N5,N10-methylene-THF -?-> dTMP + DHF
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thymidylate synthase
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pyrimidine salvage pathway:
pyrimidine + PRPP -?-> pyrimidine NMP |
pyrimidine phosphoribosyl transferase
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pyrimidine degradation:
uracil/thymine -> ? |
alanine/aminoisobutyrae + NH3 -> citric acid cycle
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adenosine deaminase deficiency
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no adenosine -> inosine
more dATP -> no ribonucleotide reducase -> no proliferation |
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allopurinol
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alloxanthin -> no xanthine oxidase
hypoxanthine/xanthine accumulate -> IMP -> no PRPP for gout |
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methotrexate
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for cancer
no dihydrofolate reductase -> no THF -> no purine/pyrimidine synthesis |
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sulfanilamide
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anti-bacterial
folate synthesis intermediate -> no THF |
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trimethoprim
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parasite/bacterial infection
no dihydrofolate reductase -> no THF |
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fluorouracil
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cancer
-> F-dUMP -> no thymidylate synthase -> no thymidine -> no DNA |
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mercaptopurine
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mercapto-nucleotide -> no PRPP amidotransferase -> no purine synthesis
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cytosine arabinoside
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ara-c -> ara-CTP -> no DNA
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acycloguanosine
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HSV
->acyclo-GTP -> no DNA |
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azidothymidine
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HIV
-> AZ-TTP -> no DNA |
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lysosomal degradation use?
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cathepsin: protease
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cystatins
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inhibit cathepsin
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cathepsin K deficiency
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autophage, bone malformation
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serpins
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serine protease inhibitors
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proteosome lid
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receptor, isopeptidase (de-ubiquitin), unfold
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protosome core
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protease
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urea cycle
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NH4 + aspartate -> urea + fumarate
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carbamoyl phosphate + orinithine -?-> citrulline
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ornithine transcarbamoylase
mitochondrial matrix |
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citrulline + aspartate -?-> arginiosuccinate
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argioniosuccinate synthase
cytosol |
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arginiosuccinate -?-> arginine + fumarate
urea cycle |
arginiosuccinate lyase
cytosol |
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serine + THF -?-> glycine + N5,N10-methylene-THF
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serine hydroxymethyl-tranferase
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N5,N10-methylene-THF -?-> N5-methyl-THF
1C metabolism |
N5,N10-methylene-THF reductase
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N5-methyl-THF + homocysteine -?-> methionine + THF
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homocysteine methyltransferase
vitamin B12 |
