Term
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Definition
| suicide inhibitor; irreversible inhibition of thmidylate synthase; chemotherapy agent |
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Term
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Definition
| sickle cell reduces rate of attacks; decreases deoxyribonucleotide production via inhibition of ribonucleotide reductase |
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Term
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Definition
| competitively inhibits dihydrofolate reductase; inhibits synthesis of DNA, RNA, thymidylates, and proteins; cancer, immune, and eptopic pregnancy treatment |
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Term
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Definition
| competitive inhibitor incorporated into cytidine; arrests tumor growth by stopping DNA synthesis early and signaling apoptosis |
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Term
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Definition
| antiviral drug; treats herpes; competitive inhibitor of guanosine kinase in viruses; further activated by the phosphorylation of the viral enzyme; inhibits DNA polymerase and synthesis |
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Term
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Definition
| antiretroviral; reverse transcriptase inhibitor; treats HIV; nucleoside analogue of guanosine; chain termination and inhibits complementary DNA enzyme (reverse transcriptase) |
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Term
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Definition
| pyrimidine analong; reverse transcriptase inhibitor; phosphorylated in T cells and other HIV target cells into its active form; only effective against retroviruses |
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Term
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Definition
| reverse transcriptase inhibitor; azide group allows it to cross BBB |
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Term
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Definition
| immunosuppressve; activated to methyl-thioinsine monophosphate by cellular enzymes; blocks purine synthesis especially effective in DNA synthesis inhibition in T and B cell lymphocytes |
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Term
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Definition
| immunosuppressive; acts early in purine synthesis to compete with guanine and hypozanthine at the IMP step (AMP or GMP pathway commitment step) |
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Term
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Definition
| xanthine oxidase inhibitor; increases exretion of more soluble xanthine or hypoxanthine |
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Term
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Definition
| xanthine oxidase inhibitor; increases exretion of more soluble xanthine or hypoxanthine |
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Term
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Definition
| inhibition of renal urate re-absorption; treatment for hyperuricemia |
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Term
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Definition
| converts uric acid to allantoin; recombinant uricase; limited to treatment of tumor lysis syndrome induced hyperuricemia |
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Term
| acute intermittent porphyria (AIP) |
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Definition
defect in porphobilinogen deaminase
PBG accumulates
increased demand for heme in liver activates the deficiency symptoms
treated with hemin infusion |
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Term
| porphyria cutanea tarda (PCT) |
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Definition
defect in uroporphyrinogen decarboxylase
uroporphyrinogen III and I accumulate
acquired deficiency
activated by alcohol abuse and hepatitis C
treated with phlebotomy and reduction of hepatic iron |
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Term
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Definition
catalyzes reduction of ribose moiety (ADP, GDP, and CDP) to deoxyribose
part of purine de novo synthesis
only works on diphosphate form
adds hydrogen and releases water |
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Term
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Definition
glycine - base
C8 - methyl-FH4
N3 - glutamine
C6 - CO2
N1 - aspartate
C2 - formyl-FH4 |
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Term
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Definition
base - aspartate
N3 - glutamine
C2 - CO2 |
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Term
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Definition
| dUMP + n5n10-methylene-tetrahydrofalte --> dTMP + dihydrofolate |
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Term
| purine synthesis regulation |
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Definition
(+) PRPP,
(-) IMP, GMP, AMP, mycophenolic acid |
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Term
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Definition
inhibits mitochondrial DNA and enzymes
causes lactic acidosis and liver failure
blocks gluconeogenesis, blocks FOX, decreased oxidation of lactate |
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Term
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Definition
Cause: deficiency of HGPRT, X-linked
S/S: failure of purine salvage, hyperuricemia, self-mutilation, mental retardation |
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Term
| Adenosine deaminase deficiency |
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Definition
Blocks iodine from adenosine reaction
Increased levels of adenosine blocks nucleotide synthesis
Affects immune cell proliferation immensely, causes immunodeficiency |
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Term
| Acute intermittent porphyria |
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Definition
Defect in pohobilinogen deaminase
Porphobilinogen accumulates
Occurs when there is increased demand for home synthesis
Treat with hemin infusion |
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Term
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Definition
Defect in uroporphyrinogen decarboxylase
Uroporphyrinogen III and I accumulate
Activated by alcohol abuse and hepatitis C
Treat with phlebotomy and hepatic iron reduction |
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Term
| ALA dehygrogenase deficiency |
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Definition
| Associated with lead poisoning |
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Term
| Eryhtropoietic protophyria |
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Definition
| Associated with lead poisonong |
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Term
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Definition
Converts cholesterol to 7-hydroxycholesterol
First step in bile salt synthesis, rate-limiting step
(+) low intracellular [bile salt]
(-) high intracellular [bile salt] |
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Term
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Definition
| Active transported of bile from hepatocyte to bile duct |
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Term
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Definition
Sodium-linked transporter in distal ileum
Intestine lumen to intestinal cell to portal blood |
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Term
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Definition
Sodium-linked transporter in liver
Transports bile from portal blood into hepatocyte |
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Term
| Increased indirect bilirubin |
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Definition
Hemolysis, increased production
Heart failure, impaired uptake
C-N or Gilbert, impaired conjugation ability |
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Term
| Increased direct bilirubin |
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Definition
Appears in urine
Liver or bile duct disease, impaired excretion
Both indirect and direct high in blood |
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Term
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Definition
decreased UDP-gluconyltransferase activity due to promoter mitatiom
stress worsens, result in jaundice and increased indirect bilirubin blood level |
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Term
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Definition
Inactive UDP-gluconyltransferase due to coding region mutation
Increased levels of unconjugated bilirubin |
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Term
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Definition
cMOAT (transporter) is defective, less transport of bilirubin into bile
Aka ABCC2, MRP2
Increased levels of conjugated bilirubin |
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Term
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Definition
Glc + NADPH --> sorbital + NADP+
Enzyme: aldose reductase
Increased pathway use with increased blood glucose
Sorbitol has osmotic effects in eyes, causes eye symptoms of DM |
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Term
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Definition
Increased activity with increased blood glucose levels
Gets rid of methylgluoxal which is dangerous but makes lactate |
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Term
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Definition
Increased activity with increased blood glucose levels
Alters glycosylation of proteins and lipids
Alters transcription activity
Might cause insulin resistance |
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Term
| Advanced glycation end products |
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Definition
Increased amounts with increased blood glucose levels
Ex: Schiff base, global, methylgloxal, fructosamine
Believed to cause blood vessel issues of DM
Very reactive and potentially damaging molecules |
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Term
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Definition
Maturity onset diabetes of the young
Monogeneticndisease, i.e. mutation in glucokinase, TFs, MODY X
Single gene mutation that results in DM |
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Term
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Definition
S/s: enlarged liver, high ALT and AST, bright liver on ultrasound, far globules on liver biopsy
Causes: alcoholic and non-alcoholic (obesity, DM2, metabolic syndrome) |
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Term
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Definition
ANI <0 --> NAFLD
ANI >0 --> ALD
ANI = -58.5 + 0.637(MCV) + 9.31(AST/ALT) - 0.406(BMI) + 6.35(MALE) |
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Term
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Definition
| Characteristics: central waist circumference obesity, dyslipidemia (TAG high, HDL low), high fasting blood glucose |
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Term
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Definition
Male: 40-60g/day; 2-3 drinks
Female: 10-20g/day; 1 drink
12-14g = 12oz beer, 4oz wine, 1.5oz spirits |
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Term
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Definition
40-50-60 to 70-80-90
40mmHg to 70% saturation
50 to 80%
60 to 90%
170 to 99% |
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Term
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Definition
Fe is oxidixed to 3+ state, cannot bind O2
Oxygen content and delivery are reduced without cardiac output signaled to increase
Pulse oximeter plateaus at 85%
Chocolate blood
Caused by drugs and oxidant stress |
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Term
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Definition
Localized to ER
Use oxygen in intermediary to metabolize drugs |
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Term
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Definition
Localized to inner mitochondrial membrane (mostly in adrenal glands and gonads)
Use oxygen for synthetic reactions (steroids, bile salts, etc.) |
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Term
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Definition
Iron(III) in p450 binds substrate
Iron is reduced (to II)
Oxygen is added to complex
Oxygen is changed to reactive form
Results in hydroxylation of substrate |
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Term
| ROS synthetic processes/enzymes |
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Definition
| NADH/NADPH oxidase, xanthine oxidase, lipoxygenase, cyclooxygenase, p450 monooxygenase, mitochondrial oxidative-phosphorylation, superoxide dismutase, Fenton reaction |
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Term
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Definition
Superoxide O2•-
Hydrogen peroxide H2O2
Hydroxyl radical OH•- |
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Term
| Sulfonylureas & meglitinides |
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Definition
Anti-diabetic drug
Stimulate insulin release by B cells
Target SUR1 subunit of K+ channel
Closes K+ channel, depolarizes B cell, opens Ca2+ channels, signals insulin secretion
Adverse: hypoglycemia, weight gain |
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Term
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Definition
Anti-diabetic drug
Inhibit intestinal a-gluconidase
Reduce glucose formation from ingested polysaccharides
Adverse: flatulence, abdominal discomfort |
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Term
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Definition
Anti-diabetic drug, insulin sensitizers
Targets PPAR-gamma, increases FA uptake, decrease lipolysis, increase insulin sensitizing factors, decrease insulin resistance factors
Adverse: weight gain |
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Term
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Definition
Anti-diabetic drug, variant of skyline of B cell
Suppresses glucagon secretion, suppresses insulin secretion, delays gastric emptying, decrease appetite and weight |
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Term
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Definition
Anti-diabetic drugs
Homolog of natural Gulp-1
Increase insulin secretion in glucose-dependent manner, decrease glucagon secretion, increase insulin sensitivity, increase B cell mass and expression of insulin gene, delays gastric emptying, increase brain satiety
Ex: Exenatide, Liraglutide, CJC-1131, Albugon |
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Term
| Enterocyte metabolism of glutamine |
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Definition
| Glutamine --> alanine + NH4+ + CO2 + energy |
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Term
| Lymphoblast metabolism of glutamine |
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Definition
| Glutamine --> NH4+ + aspartate + energy |
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Term
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Definition
takes up glutamine and some ammonium
excretes urea |
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Term
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Definition
| Capture ammonium missed by periportal cells and fix it into Glutamine using Glutamine synthetase |
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Term
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Definition
70g liver glycogen
120g muscle glycogen
20g circulating glucose
15kg adipose fat
6kg muscle protein |
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Term
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Definition
Adds glucose residues to growing glycogen (a-1,4)
(-) phosphorylation
(+) G6P, dephosphorylation |
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Term
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Definition
(-) dephosphorylation
(+) phosphorylation |
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Term
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Definition
Fasted = phosphatase form, phosphorylated
Fed = kinase form, dephosphorylated |
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Term
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Definition
(-) phosphorylation, acetyl CoA (indirect)
(+) dephosphorylation |
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Term
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Definition
(-) phosphorylation
(+) dephosphorylation, citrate, insulin |
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Term
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Definition
(-) phosphorylation
(+) dephosphorylayion, F-1,6,-bP, acetyl CoA |
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Term
| Malonyl CoA decarboxylase |
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Definition
Converts malynyl CoA back to acetyl CoA in muscles, allows FA oxidation to proceed
(-) dephosphorylation
(+) phosphorylation |
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Term
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Definition
X-linked recessive
Exacerbated by oxidant drugs, antimalarial compounds, hepatitis a/infections, and diet
Presents with weakness, fatigue, yellowing, fever, elevated indirect bilirubin, low Hb, low hematocrit, heinz bodies, hemoglobinuria |
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Term
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Definition
Abdominal pain, flatulence, diarrhea, and naseau with lactose exposure
Compromised lactase activity |
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Term
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Definition
Defect of galactose-1-phosphate uridyltransferase (GALT)
Feeding difficulty, vomiting, jaundice, hepatomegaly, hypoglycemia, CATARACTS |
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Term
| Hereditary fructose intolerance |
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Definition
Defect in aldolase B
Hypoglycemia, fructosemia, fructosuria, hyperuricemia, lactic acidosis, urine with reducing sugar, urine negative for glucose |
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Term
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Definition
Lack of fructokinase A
means unable to convert fructose to phosphate form
Symptoms due to kidney elimination of fructose
Similar to aldolase B deficiency |
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