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72 Cards in this Set
- Front
- Back
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Where do these take place
Fatty acid oxidation Acetyl-CoA production TCA cycle Oxidative phosphorylation |
Mitochondria
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Where do these take place
Glycolysis fatty acid synthesis HMP shunt Protein/steriod synthesis |
Cytoplasm
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Where do these take place
Heme synthesis Urea cycle Gluconeogenesis |
Both mitochondria and cytosol
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What is the Rate determining step for Glycolysis
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Phosphofructokinase-1 (PFK-1)
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What is the Rate determining step for Gluconeogenesis
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Fructose-1,6-bisphosphatase
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What is the Rate determining step for TCA cycle
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Isocitrate dehydrogenase
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What is the Rate determining step for Glycogen synthesis
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Glycogen synthase
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What is the Rate determining step for Glycogenolysis
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Glycogen phosphorylase
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What is the Rate determining step for HMP shunt
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Glucose 6 Phosphate dehydrogenase
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What is the Rate determining step for De novo pryimidine synthesis
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carbamoyl phosphate synthetase II
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What is the Rate determining step for De novo purine synthesis
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Glutamine-PRPP amidotransferase
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What is the Rate determining step for Urea Cycle
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Carbamoyl phosphate synthetase I
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What is the Rate determining step for Fatty acid synthesis
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Acetyl-CoA carboxylase (ACC)
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What is the Rate determining step for Fatty Acid oxidation
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Carnitine acyltransferase I
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What is the Rate determining step for Ketogenesis
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HMG-CoA synthase
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What is the Rate determining step for Cholesterol synthesis
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HMG-CoA reductase
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What are the 4 electron transport inhibitors that block and stop ATP synthesis
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Rotenone
Cyanide antimycin A Carbon monoxide |
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What is the poison that is a ATPase inhibitor
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Oligomycin
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What are 3 uncoupling agents to increase fever
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2,4-DNP
aspirin overdose thermogenin in brown fat |
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What are all the steps on the krebs cycle
**Citrate is krebs starting substrate for making oxaloacetate |
Citrate
isocitrate a-Ketogluterate Succinyl CoA Succinate Fumerate Malate Oxaloacetate |
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Gluconeogenesis irreversible enzyme
Pyruvate carboxylase |
in mitochondria
pyruvate ----- oxaloacetate |
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Gluconeogenesis irreversible enzyme
PEP carboxykinase |
In cytosol
Oxaloacetate ---- phosphoenolpyruvate |
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Gluconeogenesis irreversible enzyme
Fructose-1,6-bisphosphatase |
In cytosol
Fructose-1,6-bispohosphate ----- fructose 6-P |
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Gluconeogenesis irreversible enzyme
glucose 6-P |
in ER
Glucose 6-P ----- Glucose |
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What are all the irreversible steps in gluconeogenesis
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Pyruvate carboxylase = pyruvate ----- oxaloacetate
PEP carboxykinase = Oxaloacetate ---- phosphoenolpyruvate Fructose-1,6-bisphosphatase = Fructose-1,6-bispohosphate ----- fructose 6-P glucose 6-P = Glucose 6-P ----- Glucose |
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How is NADPH formed
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Glucose-6-P + G6PDehydrogenase = 2 NADPH (ribulose-5-Pi)
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what is Ribulose-5-Pi further reduced to
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Ribulose-5-P + Transketolases = Ribulose-6-Pi + G3P and F6P
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Fructose intolerance
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Aldolase B deficiency
causes hypoglycemia, jaundice, cirrhosis, Vomiting Causes a build op of F-1-P and depletes the pool of Phosphates |
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Essential Fructosuria
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defect in fructosekinase
benign causes fructose to be in blood and urine. |
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Classic Galactosemia
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Absence of Galactose-1-phosphate uridyltransferase
**accumulation of toxic substances "galactitol" accumulates in teh lens of the eye** Symptoms = FFT, jaundice, hepatomegaly, infant cataracts, mental retardation |
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Galactokinase deficiency
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defeciency in Galactokinase
** galactitil accumulates if galactose is present in diet. Mild condition Symptoms = galactose in urine, infantile cataracts, (failure to follow objects or smile) |
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Ketogenic amino acids
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Leucine, lysine
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What are all the substrates in the urea cycle
Ordinary careless crappers are also frivolous about urination |
Ornithine + Carbymole phosphate
makes citruline Aspartate Arginosuccinate Fumarate Arginine Urea ** carbamoyl phosphate is made VIA carbamoyl phosophate synthetase I in the mitochondria** |
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What is Hyperammonemia
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results in excess NH4 with depletes alpha-ketogluterate and stops TCA cycle
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What is the treatment for hyperammonemia
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limit protein in diet
give benzoate or phenylbutyrate (they bind and eliminate proteins) and decrease ammonia levels |
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Ornithine transcarbamoylase deciciency
(OTC) |
Cannot eliminate ammonia. cannot make citruline
**execss carbamoyl phosphate is converted to orotic acid** Find orotic acid in blood and urine and symptoms of hyperammonemia |
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What are the symptoms of hyperammonemia
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Tremor
Slurring of speech somnolence vomiting cerebral edema blurring of vision |
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Tryptophan makes what
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serotonin
melatonin Niacin ---- NAD/NADP |
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phenylalanine makes what
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Tyrosine -- thyroxine and Dopamine
melanin dopamine NE E |
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What does glycine make
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porphyrin
Heme |
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What does arginine make
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creatinine
Urea Nitric oxide |
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What does glutamate make
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GABA
Glutathione |
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Phenylketonuria
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Due to decrease phenylalanine hydroxylase or tetrahydrobiopterin cofactor.
Findings = mental retardation, growth retard, seizures, mousy odor, eczema |
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Alkaptonuria
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congenital deficiency of homogentisic Acid oxidase
Benign disease ** dark connective tissue, urine turns black on standing** May have debillitating arthralgias |
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Albinism
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Due to tyrosinase -cant make melonin
or defective tyrosine transporters (melonin cannot get there) ***can also result from a lack of migration of neural crest cells*** |
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Cystinuria
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Defect in renal amino acid transporter
cannot resorbe cystine Excess cystine can cause formation of staghorn stones |
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Hartnup disease
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tryptophan is excreted due to defective transporter and causes pellagra
**tryptophan makes niacin** |
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Von Gierkes disease
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Deficient in glucose-6-Phosphatase
** severe fasting hypoglycemia, increase glycogen in liver, increase blood lactate** |
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Pompe's disease
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Cardiomegaly and systemic findings leading to early death
Deficient in lysosomal alpha-1,4-glucosidase |
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Cori's disease
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milder form of Von Gierkes
deficient in debranching enzyme alpha-1,6-glucosidase |
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McArdles disease
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Deficient in glycogen phosphorylase
** increase glycogen in muscle but cannot break it down. painful cramps when exercizing** |
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Fabry's disease
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Peripheral neurapathy of hands/feet, angiokeratomas CV disease/renal disease
**alpha-galactosidase A** Build up of ceramide X-liked recessive |
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Gauchers disease
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Most common
Hepatosplenomegaly, aseptic necrosis of femur, bone crisis **beta-glucocerebrosidase** Accumulate glucocerebroside |
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Niemann-Pick disease
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Progressive neruodegeneration, hepatosplenomegaly, cherry-red spot on macula
**Sphingomyelinase** accumulate Sphingomyelin |
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Tay-Sachs disease
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Progressive neurodegeneration, developmental delay, cherry-red spot on macula *lysosomes with onion skin and no hepatosplenomegaly*
**Hexosaminindase A** accumulate GM2 Ganglioside |
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Krabbe's disease
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Peripherial neuropathy, developmental delay, optic atrophy, globiod cells
**Galactocerebrosidase** accumulate Galactocerebroside |
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Metachromatic leukodystrophy
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Central and peripheral demyelination with ataxia, and dementia
**Arylsulfatase A** accumulate cerebroside sulfate |
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Hurlers syndrome
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Developmental delay, gargoylism, corneal clouding, hepatosplenomegaly
**Alpha-L-iduronidase** accumulate heparan/dermatan sulfate |
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Hunters syndrome
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mild hurlers + aggressive behavior, no cornial clouding
**iduronate sulfatase** Accumulate heparan/dermatan sulfate |
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Apoprotein A
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Activats LCAT
Lethicin - cholesterolacyltransferase |
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Apoprotein B-100
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binds to LDL receptor and mediates VLDL secretion
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Apoprotein C-II
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Cofactor for lipoprotein lipase
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Apoprotein B-48
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Mediates chylomicron secretion
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Apoprotein E
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Mediates Extra (remnant) uptake
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Chylomicrons job
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Delivers dietary TG's to peripheral tissue
takes the chylomicron remnants back to the liver |
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VLDL's job
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Delivers hepatic TG's to peripheral tissue **secreted by liver**
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IDL's job
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formed in the degradation of VLDL
Delivers triglycerides and cholesterol to liver where they degrade to LDL |
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What is LDL's job
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Delivers hepatic cholesterol to peripheral tissues.
taken up via receptor mediated endocytosis |
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What is HDL's job
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picks up cholesterol from the periphery and takes it back to the liver
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Familial hypercholesterolemia
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increased LDL in blood and high cholesterol
AD disease ** absent or decreased LDL receptors** accelerated atherosclerosis |
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A-Beta-lipoproteinemia
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cannot make B-100 or B-48
Chylomicrons cannot be absorbed from gut. malabsorption issues fatty crap all fat soluble vitamin deficient |
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Hypertriglyceridemia
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overproduction of VLDL
causes pancreatitis |
