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47 Cards in this Set

  • Front
  • Back
CPS 1
location: mitochondria
pathway: urea cycle
nitrogen source: ammonia
CPS 2
location: cytosol
pathway: pyrimidine synthesis
nitrogen source: glutamine
How does UV light damage DNA?
creates thymine dimers on SAME strand of DNA
DNA pol alpha (eukaryotic)
replicates lagging strand, synthesizes RNA primer
DNA pol beta, epsilon (eukaryotic)
repairs DNA
DNA pol gamma (eukaryotic)
Replicates mitochondrial DNA
DNA pol delta (eukaryotic)
Replicates leading strand of DNA
4 ways proteins interact with DNA
1. helix turn helix
2. helix loop helix
3. leucine zipper protein
4. zinc finger motif
Metabolism site: mitochondria
Fatty acid oxidation (beta oxidation), acetyl CoA production, TCA cycle, oxidative phosphorylation, ketone synthesis
Metabolism site: cytoplasm
Glycolysis, fattay acids synetheiss, HMP shunt, protein synthesis (RER), steroid synthesis (SER)
Metabolism site: both
Heme synthesis, urea cycle, gluconeogenesis

HUGs take two
Kinases
uses ATP to add high energy phosphate group onto substrate
Phosphorylase
adds inoragnic phosphate onto substrate without using AtP
Phosphatase
removes phosphate group from substrate
Dehydrogenase
oxidizes substrate
Carboxylase
adds 1 carbon with help of biotin
Glycolysis rate limiting step
Phosphofructokinase 1 (PFK1)
Gluconeogenesis rate limiting step
Fructose 1,6 bisphosphatase
TCA cycle rate limiting step
Isocitrate dehydrogenase
Glycogen synthesis rate limiting step
Glycogen synthase
Glycogenolysis rate limiting step
Glycogen phosphorylase
HMP shunt rate limiting step
Glucose 6 phosphate dehydrogenase (G6PD)
De novo pryimidine synthesis rate limiting step
CSP II
De novo purine synthesis rate limiting step
Glutamine PRPP amidotransferase
Urea cycle rate limitng step
CSP I
Fatty acid synthesis rate limiting step
Acetyl CoA carboxylase (ACC)
Fatty acid oxidation rate limiting step
Carnitine acyltransferase I
Ketogenesis rate limiting step
HMG CoA synthase
Cholesterol rate limiting step
HMG CoA reductase
Heme synthesis rate limiting step
Aminolevulinic synthase
Bile acid synthesis rate limiting step
7 alpha hydroxylase
Von Gierke's dz (type I)
Severe fasting hypoglycemia, very high glycogen in liver, inc blood lactate, hepatomegaly, inc uric acid

Deficient enzyme: glucose 6 phosphatase
Pompe's disease (type II)
cardiomegaly and systemic findings leading to early death by age 3 (trashes the pumps)

Deficient enzyme: lysosomal alpha 1,4 glucosidase (acid maltase)
Cori's disease (type III)
Milder form of type I with normal bood lactate levels

Deficient enzyme: debranching enzyme (alpha 1,6 glucosidase)

Gluconeogenesis is intact
McArdle's disease (type V)
Inc glycogen in muscle but cannot break it down, leading to painful muscle cramps, myoglobinuria with strenuous exercise

Deficient enzyme: skeletal muscle glycogen phosphorylase
Phenylalanine derivatives
--> tyrosine (thyroxine) --> dopa (melanin) --> dopamine --> NE --> epi
Tryptophan derivatives
B6 --> niacin --> NAD/NADP

BH4 --> serotonin --> melatonin
Histidine derivatives
B6 --> histamine
Glycine derivatives
B6 --> porphyrin --> heme
Arginine derivatives
Creatine, urea, nitric oxide
Gluamate derivatives
B6 --> GABA (gluamate decarboxylase requires B6)

Glutathione
AI apolipoprotein
Activates LCAT
B 100 apolipoprotein
Binds to LDL receptor, mediates VLDL secretion, IDL
C II apolipoprotein
Cofactor for lipoprotein lipase
B 48 apolipoprotein
Mediates chylomicron secretion
E apolipoprotein
Mediates Extra (remnant) uptake
C III apoliprotein
Inhibs LPL