Nitrogen Metabolism, AA metab, Urea cycle, AA dx, Heme metab and Integration of Metabolism Flashcards
Net accumulation of proteins as in growth and pregnancy
Positive nitrogen balance
Net breakdown of proteins as in surgery, advanced cancer, kwashiorkor or marasmus, starvation
Negative nitrogen balance
Average protein turnover per day
300 to 400 g per day
Amount of protein degraded and resynthesized from AA
Protein turnover
Sum of all free amino acids in cells and ECF
Amino acid pool
First phase of amino acid catabolism:
Removal of alpha amino group, forming ammonia and alpha keto acid
Deamination
First phase of amino acid catabolism:
Removal of amino group for AA yields these 2 products
Alpha keto acid
Ammonia
First phase of amino acid catabolism:
Ammonia may be excreted as free ammonia in urine and stool, but majority is converted to _____
Urea
Second phase of AA catabolism
Carbon skeletons of alpha keto acids are converted to common intermediates of what 2 energy-producing metabolic pathways
Glycolysis
TCA
Term that denotes excretion of nitrogen among telostean fish, which excrete highly toxic ammonia
Ammonotelic
Term that denotes manner of excretion of excess nitrogen among birds, which excrete uric acid as semisolid guano
Uricotelic
Term that denotes manner of excretion of excess nitrogen among land animals including humans, who excrete non-toxic, water-soluble urea
Ureotelic
Amino acids transfer their amino group to a-kg resulting in the formation glutamate
Transamination
During transamination, amino acids transfer their amino group to a-kg resulting in the formation of _____
Glutamate
Enzyme for transamination
Aminot transferase
Co enzyme of aminotransferase
Pyridoxal phosphate
Alanine + a-kg —> _____ + ______
Glutamate, pyruvate
Aspartate + a-kg —> ____ + _____
Glutamate, OAA
2 major step in the first phase of AA catabolism
Transamination
Oxidative deamination
AA catabolism
In the liver and kidney, glutamate is oxidatively deaminated to release free ammonia
Oxidative deamination
2 organs involved in oxidative deamination
Liver
Kidney
Enzyme for oxidative deamination
Glutamate dehydrogenase
AA catabolism
In the liver and kidney, _______ is oxidatively deaminated to release free ammonia
Glutamate
AA catabolism
In the liver and kidney, glutamate is oxidatively deaminated to release _____
Free ammonia
2 AA that are used for transport of ammonia from peripheral tissues
Glutamine
Alanine
TRANSPORT OF AMMONIA FROM PERIPHERAL TISSUES
In muscle, pyruvate is transaminated to _____
Alanine
Alanine is transported to the liver where it is converted back to ______
Pyruvate
In most tissues, glutamate combines with ammonia to form ____ via the enzyme ______
Glutamine
Glutamine synthetase
Glutamine is transported in the blood and may be deaminated to release ammonia in two organs:
Liver
Kidneys
Liver releases ammonia in response to _____
High protein intake
Kidneys release ammonia in response to ______
Metabolic acidosis
Pathway for the conversion of the body’s nitrogenous waste to urea
Urea cycle
Organ site of urea cycle
Liver
Subcellular site of urea cycle
Mitochondria and cytosol
3 substrates for urea cycle
CO2, ammonia, aspartate
Product of urea cycle
Urea
Rate-limiting step for urea cycle
NH3 + CO2 —> carbamoyl phosphate
Enzyme: carbamoyl phosphate synthetase I
Allosteric activator for the rate-limiting step of urea cycle
N-acetylglutamate
Identify enzyme for this step in the urea cycle
CO2 + NH3 —> carbamoyl phosphate
Carbamoyl phosphate synthetase I
Identify enzyme for this step in the urea cycle
Carbamoyl phosphate + L-ornithine —> citrulline
Ornithine transcarbamoylase
Identify enzyme for this step in the urea cycle
Citrulline + aspartate —> argininosuccinate
Argininosuccinate synthetase
Identify enzyme for this step in the urea cycle
Argininosuccinate —> fumarate
Argininosuccinate lyase
Identify enzyme for this step in the urea cycle
L-arginine —> urea + L-ornithine
Arginase
Identify products in this step of urea cycle
Carbamoyl phosphate + L ornithine —>
Citrulline
Identify products in this step of urea cycle
Citrulline + aspartate —>
Argininosuccinate
Identify products in this step of urea cycle
Argininosuccinate —>
Fumarate + L-arginine
Identify products in this step of urea cycle
L-arginine + H20 —>
Urea, L-ornithine
2 reactions in urea cycle that require ATP
CO2 + NH3 —> carbamoyl phosphate (2 ATPs)
Citrulline + L-aspartate —> argininosuccinate (1 ATP)
How many ATPs are required to form 1 mol of urea?
3 (but 4 high energy bonds)
T/F
In urea cycle, there is no net loss or gain of ornithine, citrulline, argininosuccinate, or arginine
T
_______ functions solely as enzyme activator and regulates urea synthesis
N-acetylglutamate
2 fates of urea:
Blood—>kidney —> urine
Intestines—> urease converting bacteria convert them back to CO2 and NH3
Tremors, slurring of speech, vomiting, blurring if vision
Cerebral edema
Somnolence, coma, death
Hyperammonemia
Most common enzyme deficiency in urea cycle
Onithine transcarbomylase
Most severe enzyme deficiency in urea cycle
Carbamoyl phosphate synthetase 1 deficiency
Rx for hereditary hyperammonemia
Phenylbutyrate
It gets converted to phenylacetate, binds to glutamine, and then excreted as phenylglutamine
Dse of adults with compromised liver function
Due to cirrhosis, portal blood is shunted directly to systemic curculation
Acquired hyperammonemia
In the second phase of AA catabolism, ketogenic AA may be converted to _____, while glucogenic AA are converted to ____
Acetyl CoA or acetoacetyl CoA
Pyruvate or intermediates of TCA
Purely ketogenic AA
Lysine
Leucine
Glucogenic and ketogenic AA
Phenylalanine Tyrosine Isoleucine Tryptophan (Threonine in some textbooks)
Identify amino acid with these specialized products
Creatibe, polyamines, nitric oxide
Arginine
Identify amino acid with these specialized products
Coenzyme A, taurine, glutathione
Cysteine
Identify amino acid with these specialized products
GABA, glutathione
Glutamate
Identify amino acid with these specialized products
Heme, purines, creatine, glutathione, conjugated to bile acids, drugs, and other metabolites
Glycine
Identify amino acid with these specialized products
Histamine
Histidine
Identify amino acid with these specialized products
Carnitine
Lysine
Identify amino acid with these specialized products
S-adenosylmethionine, creatine, polyamines
Methionine
Identify amino acid with these specialized products
Sphingosine, purines, thymine
Serine
Identify amino acid with these specialized products
Serotonin, niacin, melatonin
Tryptophan
Identify amino acid with these specialized products
Catecholamines
Tyrosine
Identify amino acid with these specialized products
Thyroid hormones
Tyrosine
Identify amino acid with these specialized products
Melanin
Tyrosine
Identify amino acid with these specialized products
Serotonin
Tryptophan
Identify amino acid with these specialized products
Niacin
Tryptophan
Identify amino acid with these specialized products
Melatonin
Typtophan
Identify amino acid with these specialized products
Sphingosine
Serine
Identify amino acid with these specialized products
Thymine
Serine
Identify amino acid with these specialized products
SAM
Methionine
Catecholamine synthesis
Tyrosine—>
3,4 dihydroxyphenylalanine (DOPA)
Catecholamine synthesis
Identify enzyme and co factor
Tyrosine—>DOPA
Tyrosine hydroxylase
Tetrahydrobiopterin (THB)
Catecholamine synthesis
Identify enzyme and cofactor
3,4 DOPA—> Dopamine
DOPA decarboxylase
Vitamin B6
Catecholamine synthesis
Identify enzyme and cofactor
Dopamine—> Norepinephrine
Dopamine beta hydroxylase
Vitamin C/copper
Catecholamine synthesis
Identify enzyme and cofactor
Norepinephrine—>epinephrine
Phenylethanolamine N methyltransferase
SAM—>SAH
Catecholamine synthesis
Identify product
Dopamine —>
Norepinephrine
Catecholamine synthesis
Identify product
Norepinephrine—>
Epinephrine
Catecholamine degradation
Catecholamines are inactivated by these 2 enzymes
Monoamine oxidase (MAO) Catechol-O-methyltransferase(COMT)
Catecholamine degradation
Norepinephrine and epinephrine are degraded to _______
VMA (Vanillylmandelic acid (VMA)
Increased in pheochromocytoma
Catecholamine degradation
Dopamine is degrade into ______
Homovanillic acid (HVA)
Amino acid disorders
Enzyme deficiency in PKU
Phenylalanine hydroxylase
Or tetrahydrobiopterin
Amino acid disorders
Tyrosine becomes essential and phenylalanine accumulates
PKU
Amino acid disorder Severe mental retardation Failure to walk or talk Seizures Fair skin Eczema Musty body odor
PKU
Amino acid disorders
Accumulation of this phenylketone in PKU gives the characteristic odor to urine
Phenylpyruvate
Amino acid disorders
Dietary control for PKU aims to decrease _____ and increase ____
Phenylalanine
Tyrosine
Amino acid disorders
Congenital deficiency of this enzyme leads to alkaptonuria
Homogentisate oxidase
Amino acid disorders
In alkaptonuria, there is acumulation of this substance
Homogentisic acid
Amino acid disorder
Dark urine on standing
Dark connective tissue (ochronosis)
Benign dse but may have debilitating arthalgia
Alkaptonuria
Amino acid disorders
Tx for alkaptonuria
Reduce phenylalanine and tyrosine in diet
Vit C for older children and adults
Dietary mgt for PKU
Low protein low carb
Identify disorder
Defective melanin synthesis from tyrosine, which may be from
Defective tyrosine transporters or
Absence of copper-requiring enzyme tyrosine
Albinism
Enzyme deficiency in albinism
Tyrosinase
Absence of pigment from hair, eyes, skin
Increased risk for skin cancer
Albinism
Defect in methionine degradation keading to high plasma and urinary levels of homocysteine and methionine and low levels of cysteine
Homocystinuria
Homocystinuria leads to increased plama and urinary levels of _____ and ______ and low levels of ______
Homocysteine, methionine
Cysteine
Either of these 2 enzymes deficiency leads to homocysteinuria
Methionine synthase
Cystathionine B Synthase
Coenzyme of methionine synthase
Methylcobalamin
Coenzyme of cytathionine B synthase
Pyridoxal phosphate
Most common deficiency in homocysteinuria
Cystathionine B synthase
Ectopia lentis (downward displacement) Faulty bone development and osteoporosis Mental retardation Tendency to form thrombi Myocardial infarction Stroke in children and young adults
Homocystinuria
Vitamin supplementation for homocystinuria
Vitamin B6, B12, folate
Diet in px with homocystinuria:
Low in _____
High in _______
Low in eggs, fish, meat
High in plat based protein : soya, nuts
Cystine kidney stones —> staghorn calculi
Inherited defect of renal tubular amino acid transporter for Cystine, Ornithine, Lysine and Arginine in the PCT of kidneys
Cystinuria
Rx for cystinuria
Acetazolamide (to alkalinized the urine)
Enzyme deficiency that leads to a defect in the cinversion of methylmalonyl CoA to succinyl CoA
Methylmalonyl CoA mutase
Seizure, encephalopathy, stroke at age of 1 month to 1 year
Hypotonia, lethargy, failure to thrive, hepatosplenomegaly, monilial infections
Impaired metabolism of isoleucine, valine, threonine, methionine and odd chain FA
Methylmalonic acidemia
Tx for methylmalonic acidemia includes protein restriction of _____ g/kg/d with supplementation of ___ and ____^
0.5-1.5
L carnitine, cobalamin
Enzyme deficiency in MSUD
a ketoacid dehydrogenase complex
Branched chain amino acids whose degradation are blocked in MSUD
Leucine, isoleucine, valine
Cyclic compounds formed by linkage of four pyrrole rings through methyne (-HC) bridges
Porphyrins
Enzymes catalase, peroxidase, guanylate cyclase all utilize this protein for their synthesis
Heme
Rate-limiting step in heme synthesis
Glycine + succinyl CoA —> D aminolevulinic acid
Enzyme ALA synthase
Rate-limiting enzyme for heme synthesis and co factor
ALA Synthase
Co factor: Pyridoxine
In heme synthesis, condensation of 2 molecules of D ALA leads to _______ by enzyme ____^^
Porphobilinogen
Emzyme: ALA Dehydratase
- Zinc-containing enzyme for the formation of porphobilinogen during heme synthesis
- Its inhibitor
- ALA dehydratase
2. Lead (heavy metal ions)
Ferrochelatase + ______ —> Heme
Protoporphyrin IX
Identify enzyme and product impaired in this defect of Heme synthesis
ALA dehydratase deficiency porphyria
ALA dehydratase
Porphobilinogen
Identify enzyme and product impaired in this defect of Heme synthesis
Acute intermittent porphyria
PBG deaminase
Identify enzyme and product impaired in this defect of Heme synthesis
Congenital erythropoietic porphyria
Uropophyrinigen III cosynthase
Uroporphyrinogen III
Identify enzyme and product impaired in this defect of Heme synthesis
Porphyria cutanea tarda
Uroporphyrinogen decarboxylase
Coproporphyrinogen III
Identify enzyme and product impaired in this defect of Heme synthesis
Hereditary coproporphyria
Coproporphyrinogen oxidase
Protoporphyrinogen IX
Identify enzyme and product impaired in this defect of Heme synthesis
Variegate porphyria
Protoporphyrinogen oxidase
Protoporphyrin IX
Identify enzyme and product impaired in this defect of Heme synthesis
Erythropoietic protoporphyria
Ferrochelatase
Heme
Subcellular site of heme synthesis
Cytosol: D ALA to coproporphyrinogen III
the rest: mitchondria
2 enzymes in heme synthesis most sensitive to lead poisoning
ALA dehytrase and ferrochelatase
Increased urinary ALA and free erythrocyte porphyrins
Microcytic anemia with basophilic stippling of RBCs
Headached, memory loss
Peripheral neuropathy, claw hand, wrist drop
Nausea, abdominal pain, diarrhea
Deposits in gums, epiphyses
Lead poisoning
Most common porphyria
Porphyria cutanea tarda
Cutaneous fragility
Blistering of hands, forearms, face
Porohyria cutanea tarda
Enzyme deficiency in porphyria cutanea tarda
Uroporphyrinogen decarboxylase
Accumulation of PBG and D ALA in the urine
Neuropsychiatric symptoms
Urine darkens when exposed to light
Acute intermittent porphyria
Enzyme deficiency in acute intermittent porphyria
Hydroxymethylbilane synthase (uroporphyrinogen I synthase)
Tx for mild and severe attack of acute intermittent porphyria
Mild: high dose glucose
Severe: hematin
After 120 days, RBCs are taken up and degraded by the RES, particularly in these 2 organs
Liver spleen
Heme degradation releases this by-product
Carbon monoxide
Bilirubin is transported in the blood by binding to _____
Albumin
In the liver, bilirubin binds to intracellular proteins, particularly to ___
Ligandin
Enzyme for the formation of bilirubin diglucuronide
Bilirubin glucuronyltransferase
Step in heme degradation that is most susceptible to liver disease
Secretion of bilirubin into bile
In the gut, bilirubin —> _____ (colorless)
Urobilinogen
Intestinal bacteria oxidize urobilinogen—> ______ (brown)
Stercolin
Some urobilinogen is reabsorbed from the blood and enters the ________
Portal circulation
Remaining urobilinogen is transported by the blood to the kidney, where it is converted to _______ (yellow)
Urobilin
Enzyme deficient in crigler najjar, gilbert syndrome
Bilirubin glucuronyltransferase
_________ reaction is used to measure bilirubin in serum
Van den Bergh Reaction
In Van den Bergh reaction,
Assay with no methanol measures ____
Assay with methanol measures ______
Difference in two measures _______
Direct bilirubin
Total bilirubin
Indirect bilirubin
Defect in bilirubin uptake/conjugation/secretion
Dubin Johnson Syndrome
Secretion
Defect in bilirubin uptake/conjugation/secretion
Neonatal jaundice
Conjugation
Defect in bilirubin uptake/conjugation/secretion
Crigler Najjar
Conjugation
Defect in bilirubin uptake/conjugation/secretion
Gilbert syndrome
Conjugation
Direct/indirect hyperbilirubinemia
Biliary tree obstruction
Direct
Direct/indirect hyperbilirubinemia
Dubin Johnson Syndrome
Direct
Direct/indirect hyperbilirubinemia
Rotor syndrome
Direct
Direct/indirect hyperbilirubinemia
Hemolytic anemia
Indirect
Direct/indirect hyperbilirubinemia
Neonatal physiologic jaundice
Indirect
Direct/indirect hyperbilirubinemia
Crigler Najjar
Indirect
Direct/indirect hyperbilirubinemia
Gilbert syndrome
Indirect
Direct/indirect hyperbilirubinemia
Toxic hyperbilirubinemia
Indirect
Insulin/Glucagon
Source is Beta cells of islets
Insulin
Insulin/Glucagon
Source is alpha cells of islets
Glucagon
Insulin/Glucagon
Two polypeptide chains with 51 AA linked together by 2 disulfide bridges
Insulin
Insulin/Glucagon
Single polypeptide chain with 29 AA
Glucagon
Insulin/Glucagon
Second messenger is cAMP
Glucagon
Insulin/Glucagon
2nd messenger is tyrosine kinase
Insulin
Insulin/Glucagon
Stimulated by cholecystokinin and gastric inhibitory peptide
Insulin
Insulin/Glucagon
Stimulated by hypoglycemia
Glucagon
Insulin/Glucagon Increases Gluconeogenesis Glycogenolysis Uptake of AA Beta oxidation Ketigenesis
Glucagon
Insulin/Glucagon Increases Glucose uptake Glycigenesis Protein synthesis Lipogenesis
Insulin
Insulin/Glucagon
Inhibits gluconeogenesis
Glycogenolysis
Beta oxidation
Insulin
Insulin/Glucagon
Inhibits glycogenesis
Glucagon
3 hormones with similar effects as glucagon
Epinephrine
Cortisol GH
During fed state, the brain uses _____ as exclusive source of fuel
Glucose
During fasting, the brain uses_____ and _____ as sources of fuel
Glucose, ketone
TAG stores and FA are used by the brain for fuel
T/F
F. They cant cross BBB
During fed state, the PPP is activated in the liver and adipose to provide ______
NADPH
Majore source of energy in adipose tissue during marathon
FFA
Principal site of metabolism of BCAA
Skeletal muscle
Identify disorder
Defective melanin synthesis from tyrosine, which may be from
Defective tyrosine transporters or
Absence of copper-requiring enzyme tyrosine
Albinism
Enzyme deficiency in albinism
Tyrosinase
Absence of pigment from hair, eyes, skin
Increased risk for skin cancer
Albinism
Defect in methionine degradation keading to high plasma and urinary levels of homocysteine and methionine and low levels of cysteine
Homocystinuria
Homocystinuria leads to increased plama and urinary levels of _____ and ______ and low levels of ______
Homocysteine, methionine
Cysteine
Either of these 2 enzymes deficiency leads to homocysteinuria
Methionine synthase
Cystathionine B Synthase
Coenzyme of methionine synthase
Methylcobalamin
Coenzyme of cytathionine B synthase
Pyridoxal phosphate
Most common deficiency in homocysteinuria
Cystathionine B synthase
Ectopia lentis (downward displacement) Faulty bone development and osteoporosis Mental retardation Tendency to form thrombi Myocardial infarction Stroke in children and young adults
Homocystinuria
Vitamin supplementation for homocystinuria
Vitamin B6, B12, folate
Diet in px with homocystinuria:
Low in _____
High in _______
Low in eggs, fish, meat
High in plat based protein : soya, nuts
Cystine kidney stones —> staghorn calculi
Inherited defect of renal tubular amino acid transporter for Cystine, Ornithine, Lysine and Arginine in the PCT of kidneys
Cystinuria
Rx for cystinuria
Acetazolamide (to alkalinized the urine)
Enzyme deficiency that leads to a defect in the cinversion of methylmalonyl CoA to succinyl CoA
Methylmalonyl CoA mutase
Seizure, encephalopathy, stroke at age of 1 month to 1 year
Hypotonia, lethargy, failure to thrive, hepatosplenomegaly, monilial infections
Impaired metabolism of isoleucine, valine, threonine, methionine and odd chain FA
Methylmalonic acidemia
Tx for methylmalonic acidemia includes protein restriction of _____ g/kg/d with supplementation of ___ and ____^
0.5-1.5
L carnitine, cobalamin
Enzyme deficiency in MSUD
a ketoacid dehydrogenase complex
Branched chain amino acids whose degradation are blocked in MSUD
Leucine, isoleucine, valine
Cyclic compounds formed by linkage of four pyrrole rings through methyne (-HC) bridges
Porphyrins
Enzymes catalase, peroxidase, guanylate cyclase all utilize this protein for their synthesis
Heme
Rate-limiting step in heme synthesis
Glycine + succinyl CoA —> D aminolevulinic acid
Enzyme ALA synthase
Rate-limiting enzyme for heme synthesis and co factor
ALA Synthase
Co factor: Pyridoxine
In heme synthesis, condensation of 2 molecules of D ALA leads to _______ by enzyme ____^^
Porphobilinogen
Emzyme: ALA Dehydratase
- Zinc-containing enzyme for the formation of porphobilinogen during heme synthesis
- Its inhibitor
- ALA dehydratase
2. Lead (heavy metal ions)
Ferrochelatase + ______ —> Heme
Protoporphyrin IX
Identify enzyme and product impaired in this defect of Heme synthesis
ALA dehydratase deficiency porphyria
ALA dehydratase
Porphobilinogen
Identify enzyme and product impaired in this defect of Heme synthesis
Acute intermittent porphyria
PBG deaminase
Identify enzyme and product impaired in this defect of Heme synthesis
Congenital erythropoietic porphyria
Uropophyrinigen III cosynthase
Uroporphyrinogen III
Identify enzyme and product impaired in this defect of Heme synthesis
Porphyria cutanea tarda
Uroporphyrinogen decarboxylase
Coproporphyrinogen III
Identify enzyme and product impaired in this defect of Heme synthesis
Hereditary coproporphyria
Coproporphyrinogen oxidase
Protoporphyrinogen IX
Identify enzyme and product impaired in this defect of Heme synthesis
Variegate porphyria
Protoporphyrinogen oxidase
Protoporphyrin IX
Identify enzyme and product impaired in this defect of Heme synthesis
Erythropoietic protoporphyria
Ferrochelatase
Heme
Subcellular site of heme synthesis
Cytosol: D ALA to coproporphyrinogen III
the rest: mitchondria
2 enzymes in heme synthesis most sensitive to lead poisoning
ALA dehytrase and ferrochelatase
Increased urinary ALA and free erythrocyte porphyrins
Microcytic anemia with basophilic stippling of RBCs
Headached, memory loss
Peripheral neuropathy, claw hand, wrist drop
Nausea, abdominal pain, diarrhea
Deposits in gums, epiphyses
Lead poisoning
Most common porphyria
Porphyria cutanea tarda
Cutaneous fragility
Blistering of hands, forearms, face
Porohyria cutanea tarda
Enzyme deficiency in porphyria cutanea tarda
Uroporphyrinogen decarboxylase
Accumulation of PBG and D ALA in the urine
Neuropsychiatric symptoms
Urine darkens when exposed to light
Acute intermittent porphyria
Enzyme deficiency in acute intermittent porphyria
Hydroxymethylbilane synthase (uroporphyrinogen I synthase)
Tx for mild and severe attack of acute intermittent porphyria
Mild: high dose glucose
Severe: hematin
After 120 days, RBCs are taken up and degraded by the RES, particularly in these 2 organs
Liver spleen
Heme degradation releases this by-product
Carbon monoxide
Bilirubin is transported in the blood by binding to _____
Albumin
In the liver, bilirubin binds to intracellular proteins, particularly to ___
Ligandin
Enzyme for the formation of bilirubin diglucuronide
Bilirubin glucuronyltransferase
Step in heme degradation that is most susceptible to liver disease
Secretion of bilirubin into bile
In the gut, bilirubin —> _____ (colorless)
Urobilinogen
Intestinal bacteria oxidize urobilinogen—> ______ (brown)
Stercolin
Some urobilinogen is reabsorbed from the blood and enters the ________
Portal circulation
Remaining urobilinogen is transported by the blood to the kidney, where it is converted to _______ (yellow)
Urobilin
Enzyme deficient in crigler najjar, gilbert syndrome
Bilirubin glucuronyltransferase
_________ reaction is used to measure bilirubin in serum
Van den Bergh Reaction
In Van den Bergh reaction,
Assay with no methanol measures ____
Assay with methanol measures ______
Difference in two measures _______
Direct bilirubin
Total bilirubin
Indirect bilirubin
Defect in bilirubin uptake/conjugation/secretion
Dubin Johnson Syndrome
Secretion
Defect in bilirubin uptake/conjugation/secretion
Neonatal jaundice
Conjugation
Defect in bilirubin uptake/conjugation/secretion
Crigler Najjar
Conjugation
Defect in bilirubin uptake/conjugation/secretion
Gilbert syndrome
Conjugation
Direct/indirect hyperbilirubinemia
Biliary tree obstruction
Direct
Direct/indirect hyperbilirubinemia
Dubin Johnson Syndrome
Direct
Direct/indirect hyperbilirubinemia
Rotor syndrome
Direct
Direct/indirect hyperbilirubinemia
Hemolytic anemia
Indirect
Direct/indirect hyperbilirubinemia
Neonatal physiologic jaundice
Indirect
Direct/indirect hyperbilirubinemia
Crigler Najjar
Indirect
Direct/indirect hyperbilirubinemia
Gilbert syndrome
Indirect
Direct/indirect hyperbilirubinemia
Toxic hyperbilirubinemia
Indirect
Insulin/Glucagon
Source is Beta cells of islets
Insulin
Insulin/Glucagon
Source is alpha cells of islets
Glucagon
Insulin/Glucagon
Two polypeptide chains with 51 AA linked together by 2 disulfide bridges
Insulin
Insulin/Glucagon
Single polypeptide chain with 29 AA
Glucagon
Insulin/Glucagon
Second messenger is cAMP
Glucagon
Insulin/Glucagon
2nd messenger is tyrosine kinase
Insulin
Insulin/Glucagon
Stimulated by cholecystokinin and gastric inhibitory peptide
Insulin
Insulin/Glucagon
Stimulated by hypoglycemia
Glucagon
Insulin/Glucagon Increases Gluconeogenesis Glycogenolysis Uptake of AA Beta oxidation Ketigenesis
Glucagon
Insulin/Glucagon Increases Glucose uptake Glycigenesis Protein synthesis Lipogenesis
Insulin
Insulin/Glucagon
Inhibits gluconeogenesis
Glycogenolysis
Beta oxidation
Insulin
Insulin/Glucagon
Inhibits glycogenesis
Glucagon
3 hormones with similar effects as glucagon
Epinephrine
Cortisol GH
During fed state, the brain uses _____ as exclusive source of fuel
Glucose
During fasting, the brain uses_____ and _____ as sources of fuel
Glucose, ketone
TAG stores and FA are used by the brain for fuel
T/F
F. They cant cross BBB
During fed state, the PPP is activated in the liver and adipose to provide ______
NADPH
Majore source of energy in adipose tissue during marathon
FFA
Principal site of metabolism of BCAA
Skeletal muscle
