Biochemistry Flashcards
What is the most common intracellular buffer?
Protein
What is the most common extracellular buffer?
Bicarbonate
What is a zwitterion?
A molecule w/ one negative and one positive end
What is the isoelectric point?
The pH at which there is no net charge
What is the rate-limiting enzyme in glycolysis?
PFK-1
What is the rate-limiting enzyme in Gluconeogenesis?
Pyruvate carboxylase
What is the rate-limiting enzyme in the HMP shunt?
G6PD
What is the rate limiting enzyme in glycogenesis?
Glycogen synthase
What is the rate-limiting enzyme in ß-oxidation?
CAT-1
What is the rate-limiting enzyme in cholesterol synthesis?
HMG-CoA reductase
What is the rate-limiting enzyme in ketogenesis?
HMG-CoA synthase
What is the rate-limiting enzyme in purine synthesis?
PRPP synthase
What is the rate-limiting enzyme in pyrimidine synthesis?
Asp transcarbamylase
What is the rate-limiting enzyme in TCA cycle?
Isocitrate dehydrogenase
What is the rate-limiting enzyme in urea cycle?
CPS-1
What is the rate-limiting enzyme in Heme synthesis?
d-ALA synthase
What are the catabolic pathways that create energy?
ABC
- acetyl CoA production
- ß-oxidation
- citric acid cycle
What are the anabolic pathways that store energy?
EFGH
- ER
- Fatty acid synthesis
- Glycolysis
- HMP shunt
What are the pathways that are both anabolic & catabolic?
HUG
- Heme synthesis
- Urea cycle
- Gluconeogenesis
What does an isomerase do?
Creates an isomer
What does an epimerase do?
Creates an epimer, which differs around 1 chiral carbon
What does a mutase do?
Moves side chain from one carbon to another (intrachain)
What does a transferase do?
Moves sidechain from one substrate to another (interchain)
What does a kinase do?
Phosphorylates, using ATP
What does a phosphorylase do?
Phosphorylates, using inorganic phosphate
What does a carboxylase do?
Forms C-C bonds (w/ ATP and biotin), using CO2
What does a synthase do?
Consumes 2 substrates equally
What does a synthetase do?
Consumes 2 substrates, uses ATP
What does a phosphatase do?
Breaks phosphate bond
What does a hydrolase do?
Breaks a bond with water
What does a lyase do?
Cuts C-C bonds with ATP
What does a dehydrogenase do?
Removes H with a cofactor
What does a thio do?
Breaks S bonds
What is diffusion?
From high to low concentration
What is active transport?
Goes against concentration gradient
What is zero-order kinetics?
Metabolism, independent of concentration
What is 1st order kinetics?
Constant drug percentage metabolism over time, depends on drug
What is efficacy?
Max effect regardless of dose
(Lower w/ non-competitive antagonist)
What affects efficacy?
Vmax
What is potency?
Amount of drug needed to produce effect
(Lower w/ competitive antagonist)
What affects potency?
Km
What is Kd?
Concentration of drug that binds 50% of receptors
What is EC50?
Concentration of drug that produces 50% of maximal response
What is competitive inhibition?
Fights for active site
No change in Vmax
Potency decreases
What is non-competitive inhibition?
Binds a regulatory site
No change in Km
Efficacy decreases
Decr Vmax
What is an endothermic reaction?
Pos delta G
Consumes heat
What is an exothermic reaction?
Neg delta G
Gives off heat
Ex: spontaneous reaction
What is the peak level?
4 hrs after dose
(Too high => decrease dose)
What is the Trough level?
2 hours before dose
(Too high => give less often)
What is t1/2?
Half-life
= time it takes for the body to use half of the drug ingested
What is Von Gierke?
G6P-ase deficiency
=> hypoglycemia, hepatosplenomegaly
What is Pompe’s?
Cardiac alpha-1,4-glucosidase deficiency
=> die early
Heart failure in child
What is Cori’s?
Debranching enzyme deficiency
=> short branches of glycogen
What is Anderson’s?
Branching enzyme deficiency
=> long chains of glycogen
What is McArdle’s?
Muscle phosphorylase deficiency
=> muscle cramps w/ exercise
What is Essential fructosuria?
Fructokinase deficiency
=> excrete fructose
(Still have hexokinase)
What is fructosemia?
Fructose intolerance
(Aldolase B deficiency)
=> liver damage
What does a galactokinase deficiency cause?
Cataracts
What does Galactosemia cause?
Cataracts
Mental retardation
Liver damage
What does citrate shuttle do?
FA transport out of the mitochondria
What does the carnitine shuttle do?
FA transport into the mitochondria
What lysosomal diseases have a cherry-red macula?
Tay-Sachs (ß-hexosaminadase A deficiency)
Niemann-Pick (sphingomyelinase deficiency)
What lysosomal diseases have a Gargoyle-face?
Gaucher’s (glucocerebrosidase deficiency)
Hurler’s (alpha-L-iduronidase deficiency)
What is Tay-Sach’s?
Hexosaminidase A deficiency =>
- blindness
- incoordination
- dementia
What is Sandhoff’s?
Hexosaminidase A/B deficiency
What is Gaucher’s?
Glucocerebrosidase deficiency =>
- “wrinkled tissue paper” nuclei
- bone pain
What is Neimann-Pick?
Sphingomyelinase deficiency
=> zebra bodies
What is Fabry’s?
Alpha-galactosidase deficiency =>
- corneal clouding
- attacks baby’s kidneys
- X-linked recessive
What is Krabbe’s?
ẞ-galactosidase deficiency
=> globoid bodies
What is metachromatic leukodystrophy?
Arylsulfatase deficiency
=> childhood MS
What is Hunter’s?
Iduronidase deficiency
=> milder form
What is Hurler’s?
Iduronidase deficiency
=> severe form
What is Lesch-Nyhan?
HGRPRT deficiency =>
- gout
- neuropathy
- self-mutilation
- X-linked recessive
What do white diaper crystals suggest?
Excess orotic acid
What does biotin donate CO2 groups for?
Carboxylation
What does THF donate methyl groups for?
Nucleotides
What does SAM donate methyl groups for?
All other reactions
What is the difference b/w heterochromatin and euchromatin?
Heterochromatin = tightly coiled
Euchromatin = loose (10 nm)
What are the purines?
Adenine
Guanine
What are the pyrimidines?
Cytosine
Uracil
Thymine
CUT the pyramid
What is a silent mutation?
Changes leave the same amino acid
What is a point mutation?
Changes 1 base
What is a transition?
Changes 1 purine to another purine or pyrimidine to pyrimidine
What is a transversion?
Changes 1 purine to pyrimidine or vice versa
What is a frameshift mutation?
Insert or delete 1-2 bases
What is a missense mutation?
Mistaken amino acid substitution
What is a nonsense mutation?
Early stop codon
What does a Southern blot defect?
DNA
What does a Northern blot detect?
RNA
What does a Western blot detect?
Protein
What are the essential amino acids?
PVT TIM HALL
- Phe
- Val
- Trp
- Tyr
- Ile
- Met
- His
- Ala
- Leu
- Lys
What are the essential fatty acids?
Linolenic
What are the acidic amino acids?
Asp
Glu
What are the basic amino acids?
Lys
Arg
What are the sulfur-containing amino acids?
Cys
Met
What are the O-bond amino acids?
Ser
Thr
Tyr
What are the N-bond amino acids?
Asn
Gln
What are the branched amino acids?
Leu
Ile
Val
What are the aromatic amino acids?
Phe
Tyr
Trp
What is the smallest amino acid?
Glycine
What are the ketogenic amino acids?
Lys
Leu
What are the glucogenic & ketogenic AA?
PITT
- Phe
- Ile
- Thr
- Trp
What amino acids does Trypsin cut?
Lys
Arg
What amino acids does ß ME cut?
Cys
Met
What amino acids does acid hydrolysis denature?
Asn
Gln
What amino acids does chymotrypsin cut?
Phe
Tyr
Trp
What amino acid turns yellow on Nurhydrin reaction?
Pro
What does carboxypeptidase cut?
Left of any amino acid on the carboxy terminal
What does CNBr cut?
(Cyanogen bromide)
Right of Methionine
What does aminopeptidase cut?
Right of N terminus
What does mercaptoethanol cut?
Right of Cys, Met
What does elastase cut?
Right of Gly, Ala, Ser
What does Trypsin cut?
Arg
Lys
What does chymotrypsin cut?
Phe
Tyr
Trp
What does alpha-1-antitrypsin do?
Inhibits trypsin from getting loose
What is PKU?
No Phe -> Tyr (via PheHydroxylase)
- nutrasweet sensitivity
- mental retardation
- pale
- blond hair
- blue eyes
- musty odor
What is albinism?
No Tyr -> melanin
(Via tyrosinase)
What is maple syrup urine disease?
Defective metabolism of branched AA (Leu, Ile, Val)
=> AA leak out
What is Homocystinuria?
No homocysteine -> Cys
“COLA” stones
- Cysteine
- Ornithine
- Lysine
- Arginine
What is Pellagra?
Niacin deficiency
- dermatitis
- diarrhea
- dementia
- death
What is Hartnup’s?
No tryptophan => niacin + serotonin
Presents like pellagra’s
Can mimic corn-rich diet
What causes anterior leg bowing?
Neonatal syphilis
What causes lateral leg bowing?
Rickets
What are the names of the B vitamins?
“The Rich Never Lie about Panning Pyrite Filled Creeks”
- B1 = Thiamine
- B2 = Riboflavin
- B3 = Niacin
- B4 = Lipoic acid
- B5 = Pantothenic acid
- B6 = Pyridoxine
- B9 = Folate
- B12 = Cobalamin
What does Vit A do?
Night vision
CSF production
PTH
What does Vit B1 do?
Cofactors for:
- Dehydrogenases
- Transketolase (PPP)
What does Vit B2 do?
FAD cofactor
What does Vit B3 do?
NAD cofactor
or
NADP
What does Vit B4 do?
Glycolysis
No known diseases
What does Vit B5 do?
Part of Acetyl CoA
No known diseases
What does Vit B6 do?
Transaminase cofactor
Myelin integrity
What does Vit B9 do?
Nuclear division
What does Vit B12 do?
Cofactor for:
- homocysteine methyltransferase (HMT)
- methlyl-malonyl CoA mutase (MMM)
What does Vit C do?
Collagen synthesis
What does Vit D do?
Mineralization of bones, teeth
What does Vit K do?
Clotting factors
What does biotin do?
Carboxylation
(= cofactors of carboxylases)
What does Calcium do?
Neuronal function
Atria depolarization
SM contractility
What does Cu(2+) do?
Collagen synthesis
What does Fe(2+) do?
Hb function
Electron transport
What is Bronze pigmentation?
Fe deposit in skin
What is Bronze cirrhosis?
Fe deposit in liver
What is Bronze diabetes?
Fe deposit in pancreas
What is hemosiderosis?
Fe overload in bone marrow
What is hemochromatosis?
Fe deposit in organs
What does Magnesium do?
PTH and kinase cofactor
What does Zinc do?
Taste buds
Hair
Sperm function
What does Chromium (Cr) do?
Insulin function
What does Molybdenum do?
Purine breakdown
(Cofactor for xanthine oxidase)
What does manganese (Mn) do?
Glycolysis
What does Selenium (Se) do?
Heart function => dilated cardiomyopathy
What does tin (Sn) do?
Hair
What is Kwashiorkor?
Malabsorption
Big belly (ascites)
Protein deficiency
What is Marasmus?
Starvation
Skinny
Calorie deficiency
Where does the Pre- label send stuff to?
RER
prE = rEr
Where does the Pro- label send stuff to?
Golgi
prO = gOlgi
Where does the mannose-6P send stuff to?
Lysosome
Where does the N-terminal sequence send stuff to?
Mitochondria
What are the 4 types of collagen?
SCAB
- Type I: Skin, bone
- Type II: Connective tissue, aqueous humor
- Type III: Arteries
- Type IV: Basement membrane
How does Scleroderma present?
Tight skin
How does Ehler’s Danlos present?
Hyper-stretchable skin
How does Marfan’s present?
Hyperextensible joints
Arachnodactyly
Wing span longer than height
Aortic root dilatation
Aortic aneurysm
Mitral valve prolapse
Dislocated lens from bottom of eye -> look up
How does homocystinuria lens present?
Dislocated lens from top -> look down
How does kinky hair disease present?
Hair looks like copper wire
(= copper deficiency)
How does scurvy present?
Bleeding gums
Bleeding hair follicles
How does Takayasu arteritis present?
Asian female with very weak pulse
How does osteogenesis imperfecta present?
Shattered bones
Blue sclera