-
Which Sphingolipidosis produce cherry red macula?
- G1 gangliosidosis
- Tay-Sacchs
- Sandoff
-
What enzyme is deficient in GM1 gangliosidosis?
What substrate accumulates
- Beta galactosidase
- GM1 ganglioside
-
What enzyme is deficient in Tay-Sacchs?
What substrate accumulates?
- N-acetyl hexosaminidose
- GM2 ganglioside
-
What enzyme is deficient in Sandoffs?
What substrate accumulates?
- N-acetylhexosaminidase A and B
- GM2 ganglioside and globoside
-
In what sphingolipidosis are pain in lower extremities, reddish purple rash, and treatment with enzyme replacement possible?
Fabry's
-
Which sphingolipidoses produces lipid laden(crumpled tissue paper) appearance?
Gauchers
-
What local molecule released by active platelets promotes platelet aggregation and constriction of blood vessels in the area of injury?
Thromboxane A2
-
Vascular endothelial cells produce what compound that prevents platelet aggregation and vasodilates blood vessels?
Prostacyclin(PGI2)
-
PGF2 is a constrictor or dilator of blood vessels?
Constrictor
-
PGE2 is a vaso- constrictor or dilator?
Dilator
-
What enzyme produces Prostoglandins and Thromboxane A2?
Cyclooxygenase
-
What enzyme produces Leukotrienes?
Lipooxygenase
-
What are the precursors to prostoglandins, thromboxanes and leukotrienes?
Linoeic acid(18 carbons)----arachidonic acid( 20C)
-
Conversion of Arachidonic acid to prostoglandins is done by what enzyme?
PG endoperoxide synthase (PGES)
-
What are the two isoenzymes of PG endoperoxide Synthase?
COX-1 and COX-2
-
What is the difference between COX 1 and COX 2?
- COX 1 is expressed constitutively abundantly and is involved in maintenance of gastric mucosa, renal homeostasis and platelet aggregation
- COX 2 is inducible involved in in heat pain and inflammation
-
What enzyme converts membrane lipid( phosphotidylcholine) to arachidonic acid?
Phospholipase A
-
What prevents the action of phospholipase A2 and COX-2?
Glucocorticoids
-
What effect does aspirin have on platelet aggregation and cox 1?
irreversibly binds COX-1 and reduces platelet aggregation
-
What drug inhibits only COX-2?
Celebrex
-
What acts as a neutrophil chemotactic agent?
LTB4
-
Leukotrienes LTC4, D4, E4 function to do what?
- increase vascular permeability
- bronchoconstriction
- vasocontriction
- contraction of smooth muscle
-
Endoperoxides give rise to what 3 distinct group of signalling molecules?
- Prostocyclin--inhibit platel gatherin, vasodilation
- Prostoglandin---pain, uterine tone, temperature
- Thromboxanes- promote platelet aggregation, vasoconstriction
-
Heparin inhibits synthesis of what factors?
thrombin, 9,10,11,12
-
Warfarin inhibits the synthesis of what clotting factors?
Protein C, S, Vitamin K derived clotting factor--2,7,9,10
-
What is the rate limiting step for FA synthesis?
Acetyl coa Carboxylase
-
What is the rate determining enzyme for FA oxidation?
Carnitine acyl transferase I
-
Rate limiting step for HMP shunt?
G6PD--glucose 6 phosphate dehydrogenase
-
Rate limiting step for TCA?
isocitrate dehydrogenase
-
Rate limiting step for glycolysis?
PFK-1
-
Rate limiting step for cholesterol synthesis?
HMG coA reductase
-
What is the rate limiting step for glycogen lysis?
glycogen phosphorylase
-
Rate limiting step for gluconeogenesis?
Fructose 1,6 bisphosphatase
-
Rate limiting step for glycogen synthesis?
glycogen synthase
-
Why doesnt glucokinase have an allosteric inhibitor?
- because it has a high Km/low affinity for glucose
- acts as a glucose buffer in the blood
-
Which glucose transporter is insulin sensitive?
GLUT-4
-
Which GLUT is needed for fructose transport?
GLUT-5
-
What is the structure of Acetyl coA Carboxylase?
Dimer
-
Desaturation of fatty acids occurs where?
ER
-
What 2 components are used for desaturation of FA?
-
On the glycerol back bone what is the nature of FA on C1
C2
C3
- C1 saturated FA
- C2 Unsaturated FA
- C3 sat or unsaturated
-
What is the source of NADPH for FA synthesis?
- 2 NADPH from HMP shunt
- 1 NADPH from malate to pyruvate
-
Fatty acid synthase produces what kind of FA?
Palmitate
-
What inhibits carnitine acyltransferase I?
Malonyl coA
-
How does liver synthesize Glycerol 3 Phosphate?
Using glycerol Kinase
-
How does Adipose tissue synthesize glycerol 3 phosphate
Glucose is broken down to dihyroxyacetone phosphate which is reduced to glycerol 3 phosphate
-
What is the activator of lipoprotein lipase?
apoprotein CII
-
Why cant adipose tissue synthesize TAG when insulin is slow?
insulin is needed for glucose uptake which is then converted to Glycerol 3 phosphate
-
What are the two enzymes that produces NADPH?
- Glucose 6 phosphate dehydrogenase
- Malic enzyme
-
Conversion of malate to pyruvate is done by what enzyme?
Whats produced?
- Malic enzyme
- NADPH is produced
-
What enzyme converts pyruvate to OAA?
pyruvate carboxylase
-
Addition of fatty acyl coA to glycerol 3 phosphate produces what?
Phosphatidic acid
-
Phosphatidic acid is converted to diacylglycerol by what enzyme?
phosphatase
-
What enzyme removes FA's from C1 and C3 of TAG's?
Hormone sensitive lipase (HSP)
-
What enzymes phosphorylate and thus induce the action of hormone sensitive lipase?
Glucagon, Epinephrine, Cortisol??, Thyroxine?, GH??
-
Glycerol released from TAG's cannot be used because?
Adipose tissue lacks glycerol kinase so it is instead transported to the liver
-
Upon hydrolysis from TAG's, how are FA activated?
By conversion to fatty acyl coA
-
What is the rate limiting enzyme of FA beta oxidation?
Carnitine acyltransferase
-
Fatty acyl coA enzyme that activates FA? is located where?
Outer mitochondrial membrane
-
Carnitine acyltransferase converts fatty acylcoA to what product?
Fatty acyl carnitine
-
Carnitine acyltransferase I (CPT I) is located where?
in the outer mitochondrial membrane
-
What happens to fattyacyl carnintine that is formed in the intermembranous space?
It is translocated into the mitochondrial matrix
-
What enzyme converts fattyacylcarnitine to fatty acyl coA in the mitochondrial matrix?
Carnitine acyltransferase II
-
What happens to the carnitine that is released in the mitochondrial matrix from the enzymatic action of Carnitine acyltransferase?
it is translocated back to the intermembraneous space
-
What are the sources of carnitine?
-
What chain length fatty acids need carnitine for transport into the mitochondrial matrix?
LCFA
-
CAT I deficiency primarily affects what organ?
Liver
-
CAT II deficiency primarily affects what organs?
skeletal muscle and cardiac muscle
-
Medium chain acyl coA dyhydrogenase (MCAD) deficiency is a deficiency of what enzyme?
acyl coA dehydrogenase
-
What kind of FA's provide a glucogenic intermediate?
Odd chain Fatty acids
-
Why does only Odd chain fatty acids provide a glucogenic intermediate?
Because after beta oxidation, a 3 carbon molecule(propionyl coA) remains which is converted to succinyl coA
-
Methylmalonyl coA Mutase is involved in the conversion of priopionyl CoA to succinyl coA. What is the necessary coenzyme?
Vitamin B12
-
Vitamin B12 is needed for what processes?
myelination, RBC maturation, oxidation of Odd length chain fatty acids
-
What are the products of one round of beta oxidation on a FA?
-
Methylmalonic acidemia and aciduria can be caused by what factors, what are they?
- Vit B12 deficiency
- Methylmalonyl coA mutase
-
Which one is glucogenic, propionyl coA or acetyl coA?
Propionyl coA
-
Oxidation of unsaturated FA may require what high energy molecule?
NADPH
-
Failure to target VLCFA to peroxisome or peroxisome insufficiency results in what syndrome?
Zellweger syndrome
-
Failure to transport VLCFA to Peroxisome results in what disorder?
Adrenoleukodystrophy
-
What high energy molecule is used in beta oxidation in peroxisomes which produces the hydrogen peroxide radical?
FAD
-
Refsum's disease results from deficiency of what enzyme?
what accumulates?
- alpha hydroxylase
- phytanic acid
-
What are the 3 ketone bodies?
- acetoacetate
- 3 hydroxybutarate
- acetone
-
Ketone body production occurs where?
in the liver
-
Under what condition does ketone body formation occurs?
- high TAG in the blood
- Starvation
-
Why are ketone bodies produced?
- oxaloacetate is shunted to gluconeogenesis
- acetyl coA cant form citrate with OAA
- acetyl coA is diverted to form ketone bodies
-
Ketone bodies are made from what?
HMG coA
-
How are HMG coA made?
- acetyl coA+ acetyl coA= acetoacetyl coA
- acetoacetyl coA + acetyl coA= HMG coA
-
Which ketone body is not metabolized?
Acetone
-
What gives rise to acetoacetate?
HMG
-
What is hypoketotic hypoglycemia?
low levels of ketone bodies and hypoglycemia
-
Which ketone body is readily usable?
Acetoacetate
-
Excretion of ketone bodies in urine causes what condition?
Polyuria
-
What activates LCAT?
apoprotein A1
-
Ammonia is converted to what?
Urea
-
In what situations is positive nitrogen balance?
-
When is negative nitrogen balance seen?
-
What types of protein are stable?
unstable?
- structural proteins (e.g)--Hb, collagen
- unstable---regulatory enzymes
-
HB 27 marker on MHC predisposes individuals to what condition?
Ankylosing spondylitis or other inflammatory conditions
-
Cholesystokinin and secretin are secreted in response to what in the stomach?
protein
-
Zymogens are released to prevent what?
autodigestion
-
Why is digestion not normal in cystic fibrosis?
hepatopancreatic duct is clogged up with thick secretions so no enzymes reach the intestine--poor digestion
-
Kwashiorkor
- protein deficiency
- sufficient calories
-
Marasmus
- protein deficiency
- calorie deficiency
- severe muscle wasting
-
What converts pepsinogen to pepsin?
H + in the stomach
-
What converts trypsinogen to trypsin?
enteropeptidase (enterokinase)
-
What are serine proteases?
protein digesting enzymes
-
Trypsin cleaves what a.a?
-
Chemotrypsin cleaves what?
hydrophobic or acidic AA
-
Elastase cleaves what?
- A.A with small side chains
- ala, gly. ser
-
What are the 3 exopeptidases
- Carboxypeptidase A, B
- Aminopeptidases
-
How is the transport of A.A differ across the luminal and serosal surface?
- lumen-- Na+ cotransport ATP used
- serosa-- facilitated diffusion
-
Deficiency of neutral amino acids transport causes?
Hartnup
-
Cystinuria or dibasic aciduria is caused by a deficiency of what a.a?
cystine, ornithine, lysine, Arginine
-
Dicarboxylic aciduria is caused by a deficiency of what a.a?
glutamate, aspartate
-
Joseph's syndrome or glycinuria is caused by?
gly, pro, hyprolinen
-
Hartnup has what conditions?
- Dementia
- Diarhea,
- Dermatitis
-
Cystinuria occurs bc of faulty transport of a.a where?
epithelia brush border of sm. intestine and renal tubules
-
Excess cysteine in blood can lead to ?
Kidney stones
-
In a transamination, what new a.a is formed?
glutamine
-
During starvation, glutamate is converted to what?
alpha keto glutarate
-
Peroxisomes have what amino acid oxidase?
- L-amino acid oxidase
- source of hydrogen peroxide
-
D-amino acid oxidase is used to degrade proteins from what source?
Plant
-
During Transamination, all nitrogen is tunneled into what a.a?
Glutamate
-
Alpha amino group is moved from what to what?
donor amino acid to an acceptor alpha keto acid
-
What vitamin is needed for transamination?
pyridoxal phosphate B6
-
Aminotransferases exist for all amino acids except what?
-
Apartate amino transferase and alanine aminotransferase are clinically important, why?
Indicates hepatic disease
-
What enzymes would be present in hepatic cell injury?
-
Gamma glutymal transferase
alcoholic liver cirhosis
-
Cholestatic lesion is caused by?
bile duct obstruction
-
Alkaline phosphatase is a marker for what two conditions?
- cholestatic lesions
- bone disease
-
Non-oxidative deamination is seen in what two a.a deamination?
-
Glutamate dehydrogenase utilizes what two high energy carriers?
-
Glutamate dehydrogenase converts glutamtate to what ?
alpha ketoglutarate and ammonium ion
-
Xanthine oxidase produces what?
hydrogen peroxide
-
Urea cycle takes place where?
Liver
-
The nitrogen atoms in urea come from what 2 sources?
-
The Carbon in urea comes from ?
HCO3-
-
Carbamoyl Phosphate I is involved in what cycle?
Urea
-
What is the rate limiting step of the urea cycle?
Carbamoyl Phosphate synthetase I
-
Formation of ornithine takes place where in the cell?
mitochondria
-
After citrulline is formed in the mitochondria what happens to it?
it enters the cytoplasm
-
What is the allosteric activator of Carbamoyl phosphate synthetase I?
N-acetylglutamate
-
How is nitrogen amount in the blood measured?
amount of nitrogen divided by the blood's urea (e.g.......14/60)
-
What is used to screen for kidney function?
BUN and creatine
-
Wilson's disease?
- Copper accumulation in the liver
- causes chorea form movements
- caudate deposition of copper
-
Kwashiokor condition results from protein deficient MEAL?
What does the MEAL stand for
- Malnutrition
- Edema
- Anemia
- Liver change (fatty) decreases lipoprotein synthesis
-
What converts pepsinogen to pepsin?
H+
-
What converts Trypsinogen to trypsin?
enteropeptidase (enterokinase)
-
Deficiency of absorption of neutral a.a like tryptophan results in what condition?
- Hartnup (Pellagra)
- Dementia
- Diarhea
- Dermatitis
-
Which a.a are not absorbed in the PCT of the kidney and as a result cause Cystynuria?
- cysteine
- Ornithine
- Lysine
- Arginine
-
What a.a is present in excess in the kidney in Cystynuria?
Cysteine
-
Absorption deficiency of cysteine in Cystynuria can cause what?
Kidney stones
-
Defective neutral a.a transporter on kidney tubules and intestinal cells causes what condition?
Hartnup disease
-
Which a.a undergo non-oxidative deamination?
-
Upon deamination, aspartate becomes what?
oxaloacetate
-
Aminotransferases exist for all amino acids except 2, what are they?
-
In a transamination reaction,what does
aspartate become?
alpha ketoglutarate become?
- aspartate---> oxaloacetate
- alpha ketoglutarate----> glutamate
-
Transaminases use what cofactor?
pyridoxal phosphate (Vitamin B6)
-
In a transamination rxn, what does
alanine become
alpha ketoglutarate
- alanine--> pyruvate
- alpha ketoglutarate---> glutamate
-
What a.a give rise to alpha keto glutarate?
- Histidine
- Glutamate
- Arginine
- Proline
-
Urinary levels of FIGLu would be high bc of a deficiency of what?
Folate
-
What a.a give rise to pyruvate?
- Glycine
- Alanine
- Serine
- Cysteine
- Threonine
-
Phenylalanine hydroxylase uses what coenzyme?
tetrahydrobiopterin
-
Sources of succinyl coa
- Threonine
- Isoleucine and valine
- Methionine
-
What accounts for the high mitochondrial mutation rate?
No proof reading capabilities
-
Any gene located in nucleus produces what pattern?
Mendelian inheritance
-
Homoplasmy
Identical mitochondria population
-
An example for reduced penetrance
Split hand deformity
-
Example of variable expression
neurofibromatosis
-
mitochondrial mutations primarily produce what clinical disease?
neuromuscular diseases
-
Pearson syndrome affects what type of tissues?
tissue that depend on oxidative phosphorylation
-
What two coenzymes are needed for SAM regeneration?
-
What is the most soluble porphyrin?
- Uroporphoryin III
- coproporphyrin III
-
Which porphyrin is not important for synthesis of heme?
porphyrin
-
Porphyrin I is elevated in what condition?
Porphyria
-
Heme containing proteins is called what?
hemoproteins
-
Heme proteins are also present where?
- myoglobin
- cytochrome
- Catalase
- Tryptophan Pyrrolase
-
All porphyrins have a characteristic absorption spectrum in what?
visible and ultraviolet light
-
400-700 nm is what light?
visible light
-
What is cancer phototherapy?
- tumor take up porphyrin
- tissue is irradiated with UV light bc porphyrin absorb UV
-
Biosynthesis of Heme?
Liver and bone marrow
-
Heme synthesis occurs where in the cell?
-
What are 3 processes that take place in mitochondria and cytosol?
- gluconeogenesis
- urea cycle
- heme synthesis
-
What are the precursors of heme?
-
How many molecules of glycine and succinyl coA are needed for 1 molecule of heme?
8
-
What TCA cycle intermediate is used for heme synthesis?
Succinyl coA
-
how many molecules of acetyl coa are needed to form cholesterol?
18
-
What is the rate determining step of heme synthesis?
ALA synthase
-
What coenzyme is needed for ala synthase
-
2 molecules of alpha alanine combine to form what?
porphobilinogen
-
What enzyme forms porphobilinogen?
ALA dehydratase
-
1 porphobilinogen + 3 porphobilinogens coupling is catalyzed by what enzyme?
- hydroxymethylbilane synthase
- uroporphyrinogen I synthase
- Porphobilinogen deaminase
-
What converts hydroxymethylbilane to uroporphyrinogen II?
Uroporphyrinogen II synthase
-
Uroporphyrin is made from what?
Uroporphobilinogen
-
What inhibits ALA synthase?
Heme
-
ALAS1 ia present where?
ALAS2 is present where?
-
At what molecular level does heme control the ALAS1 enzyme?
-
What is hemin?
oxidized (ferric) form of heme
-
Hemin is found where?
methemoglobin---> chocolate cyanosis
-
What sugar inhibts heme synthesis?
Glucose
-
Drugs have what effect on ALA synthase?
they upregulate ALA synthase
-
Why would drugs upregulate ALA synthase?
cytochrome 450 uses heme and is involved in drug metabolism
-
Lead inhibits what 2 enzymes?
- ALA dehydratase
- Ferrochelatase
-
Lead poisoning causes what?
Anemia
-
Porphyrias are caused by?
low heme presence
-
All but one porphyrias are inherited in what manner?
Autosomal Dominant
-
Porphyrias could be of 2 kinds?
What are they?
-
Acute type of porhyria is characterized by what?
- abominal pain
- neuropsychiatric symptoms
-
What are the symptoms of cutaneous type of porphyria?
- photosensitivity
- cutaneous lesions
-
Acute intermittent porphyria
- Uroporphobilinogen I synthase
- Porphobilinogen deaminase
-
Congenital erythropoietic Porphyria is caused by what?
uroporphobilinogen III synthase
-
Uroporphyrin I is found in what type of porphyria?
Congenital erythropoietic porphyria
-
Protophyria caused by a deficiency of what enzyme?
Ferrochelatase
-
What diminshes photosensitivity?
beta carotene
-
X linked sideroblastic anemia is caused by what?
Deficiency of ALA synthase 2
-
What accumulates in Refsum disease?
phytanic acid---branched chain FA
-
What is the defect in Wilson disease?
copper transport
-
Kayser-Fleisher ring is seen in what condition?
Wilson
-
Treatment for Wilsons desease
penicillamine
-
Cause for Menke's Syndrome
Efflux of copper
-
What 2 acids undergo non oxidative deamination?
-
What two a.a do not have transaminases?
-
Oxidative deamination is catalyzed by what enzyme?
glutamate dehydrogenase
-
D amino acid oxidase uses what high energy coenzyme?
FAD
-
What are the 2 ways of transporting ammonia?
- glutamine
- alanine from pyruvate
-
Amino acid oxidases rxn take place where in the cell?
peroxisomes
-
Glutamate dehydrogenase (oxidative deamination) takes place where in the cell?
mitochondria
-
Formation of alanine from pyruvate is done by what enzyme?
Alanine transferase
-
The 2 nitrogens and 1 carbon in urea come from where?
- N-aspartate via glutamate
- N--free ammonium ion
- C--carbon dioxide
-
How many ATP's are used in Urea cycle?
4
-
Argininosuccimic acidemia is caused by a deficiency of what enzyme?
Arginosuccinate lyase
-
What substrate accumulates in Fructose intolerance?
fructose-1-phosphate
-
Fructose intolerance is caused by deficiency of what enzyme?
aldolase B
-
Essential fructosuria is caused by a deficiency of what enzyme?
Fructokinase
-
Classic galactosemia is caused by what?
galactose 1 phosphate uridyltransferase
-
Glucose 6 phosphatase is found where in the cytosol?
What organ?
ER in the liver
-
-
Accumulation of fructose 1 phosphate inhibits what pathway?
- Gluconeogenesis
- Glycogenlysis
-
High levels of galactose and/or fructose result in what condition?
- change to galacticol and fructisol
- which are osmotically active
-
High levels of ammonium ion have what effect on the TCA cycle?
it depletes alpha ketoglutarate inhibiting TCA cycle
-
What is the only phospholipid with antigenic properties?
cardiolipin
-
Plasmalogens are found where?
nerve tissue
-
Cardiolipin is found where?
Inner mitochondrial membrane
-
Sphingomyelin has what backbone?
amino alcohol
-
Which glycosphingolipids are common in membranes?
Galactocerebrosides
-
Paroxysmal Nocturnal Hemoglobinuria is caused by?
PIGA gene which is needed for synthesis of GIP anchors
-
What is a symptom of Paroxysmal Nocturnal Hemoglobinuria?
Hemolytic Anemia
-
What phospholipid is deficient in Paroxysmal Nocturnal Hemoglobinuria?
Phosphatidylinositol
-
What are the 3 types of Glycosphingolipids?
- Globosides- N acetyl galactosamine
- Cerebroside
- Ganglioside--NANA
-
What degrades Phospholipids?
Phospholipases
-
Glycerophospholipids are synthesized from where?
- Glycerol 3 phosphate
- phosphatidic acid
-
Phosphatidylinositol is broken down into what two components?
IP3 and DAG
-
Digestion of lipids begins where?
Using what enzyme?
-
What are two enzymes that degrade short to medium chain fatty acids?
Where do they act?
- Gastric lipase
- Lingual Lipase
Stomach
-
What is the structure of bile salts?
sterol ring with taurine or glycine attached to it
-
Increasing chain unsaturation does what to melting point?
it decreases it
-
Which FA is needed for prostoglandin synthesis if arachidonic acid is not present?
Linoleic
-
The R1, R2, R3 is what in a TAG?
- R1-saturated
- R2-unsaturated
- R3-either
-
Human milk is rich in what TAGs?
Short and medium length fatty acids
-
Pancreatic lipase converts TAG's to what?
- 2 monoacylglycerol
- 2 free fatty acids
-
What activates Lipase?
Trypsin
-
What does orlistat do?
inhibits pancreatic lipase secretion
-
C2 FA of phosphotidylcholine is removed by what enzyme?
C1?
- C2---phospholipase A2
- C1-Lysophospholipase
-
What hormone stimulates the contraction of gall bladder?
Release of pancreatic enzyme?
Inhibits gastric emptying?
CCK
-
Which FA do not need miscelles for transport across SI Wall?
short and medium chain
-
Where in the enterocytes are TAG resynthesizes?
ER
-
How are TAG resynthesized?
FA are activated by thiokinase and added to 2-monoglycerol
-
After cholesterol and FA are resynthesized in the ER what happens to them?
they are packed into a chylomicron with apo B48
-
Type I hypercholesterolemia is caused by what?
deficiency of Lipoprotein lipase or its activator apo protein C II
-
What leads to Familial Hyperlipoproteinemia type III?
deficiency of uptake of chylomicrons remnants
-
What a.a is used to form N-glycosidic bonds in glycoproteins?
Asparagine
-
FIGLu excretion in urine is a symptom of deficiency of what coenzyme?
Folate
-
What acts as a high energy reserve molecule in the muscle?
Creatine phosphate
-
Non ketotic HyperGlycynemia is caused by what?
- deficient glycine degradation
- high glycine in blood
-
Deficiency of what enzyme causes non ketotic Hyperglycenemia?
Glycine cleavage complex
-
Primary Hyperoxaluria caused by?
increased conversion of glycine to oxalate
-
Glycinuria is caused by?
defective absorption of glycine in kidney
-
Biliverdin thats bound to albumin dissociates from albumin in the liver and binds to what?
ligandin
-
What is needed for the conversion of heme to biliverdin?
NADPH, O2 and Fe
-
Biliverdin formation takes place where in the cell?
ER
-
Salicylates and sulfanamides have what effect on the albumin-bilirubin complex?
it dissociates it, freezing bilirubin which can cross BBB and cause kernicterus
-
What is the rate limiting step in bilirubin excretion?
MOAT
-
What does MOAT do?
Transports bilirubin into canaliculi
-
What drug increases the synthesis of UDP-Glucoronyl transferase?
Phenobarbital
-
What is Gilberts syndrome?
result?
- Partially deficient UDP glucuronyl transferase enzyme
- deficient uptake of bilirubin
- unconjugated bilirubin
-
Crigler-najjar syndrome?
result
- Deficient UDP glucuronyl transferase
- unconjugated bilirubin
-
Dubin Johnson and Rotor syndrome are what?
deficient excretion of conjugated bile
-
Alkaline phosphatase is a marker of what two physiological sites?
-
What is the direct reacting bilirubin?
conjugated one
-
What is the indirect reacting Bilirubin?
unconjugated
-
Which drug has combined effects of pharmokinetics and dynamics?
Warfarin
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