Biochem Flashcards
What is the most common intracellular buffer?
Protein
What is the most common extracellular buffer?
Bicarbonate
What is a Zwitterion?
A molecule with one negative and one positive end
What is the isoelectric point?
The pH at which there is not net charge
What is the rare limiting enzyme in Glycolisis?
PFK-1 (phospho fructokinase 1)
What is the rate limiting enzyme in Gluconeogenesis?
Pyruvate carboxylase
What is the rate limiting enzyme in the HMP shunt?
G-6PD (Glucose - 6 -phosphate dehydrogenase)
What is the rate limiting enzyme in Glycogenesis?
Glycogen synthase
What is the rate limiting enzyme in Glycogenolysis?
Glycogen phosphorylase
What is the rate limiting enzyme in Fatty Acid synthesis?
AcCoA carboxylase
What is the rate limiting enzyme in B-oxidation?
CAT-1
What is the rate limiting enzyme in cholesterol synthesis?
HMG CoA reductase
What is the rate limiting enzyme in Ketogenosis?
HMG CoA synthase
What is the rate limiting enzyme in Purine synthesis?
PRPP synthase
What is the rate limiting enzyme in Pyrimidine synthesis?
Asp transcarbamoylase
What is the rate limiting enzyme in TCA cycle?
Isocitrate dehydrogenase
What is the rate limiting enzyme in the urea cycle?
CPS I
What is the rate limiting enzyme in heme synthesis?
delta-ALA-synthase
What are the catabolic pathways that create energy?
“ABC”
Acetyl-CoA production
b-oxidation
citric acid cycle
What are the anabolic pathways that store energy?
"EFGH" ER Fatty Acid synthesis Glycolysis HMP shunt
What are the anabolic + catabolic pathways?
“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 sidechain 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 Phosphorylase do?
Phosphorylates using Pi
What does Carboxylase do?
Forms C-C bonds (w/ ATP and biotin)
What does a Synthase do?
Consumes 2 substrates
What does Synthetase do?
Consumes 2 substrates, uses ATP
What does a phosphatase do?
Breaks phosphate bond
What does a Hydrolaxe do?
Breaks a bond with water
What does a Lyase do?
Cuts C-C bonds w/ATP
What does dehydrogenase do?
Removes H with a cofactor
What does a Thio do?
Breaks S bond
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 first order kinetics?
Constant drug percentage metabolism over time, depends on drug concentration
What is Efficacy?
Max effect regardless of dose (lower w/non competitive antagonist)
What effects efficacy?
Vmax
What is Potency?
Amount of drug needed to produce effect (lower w/ comp antagonist)
What affects Potency?
Km
What is Km?
Concentration of drug that produces 50% of receptors
What is EC50?
Concentration of drug that produces 50% of maximal response
What is competitive inhibition?
Fights for active site, no DeltaVmax, potency decreases
What is Non-competitive inhibition?
binds a regulatory site, no DeltaKm, efficacy decreases, decreases Vmax
What is Endothermic Reaction>
Consumes heat
What is Exothermic Reaction?
Gives of heat
What is the Peak level?
- 4 hrs after dose (too high => decrease dose)
What is the Through level?
2hrs before dose (too high=> gives less often)
What is t1/2?
Half life, the time it takes for the body to use half of the drug ingested
What is von Gierke?
G-6Pase deficiency=> hypoglycemia, hepatosplenomegaly
What is Pompe’s?
Cardiac alpha-1, 4-glucosidase deficiency => DIE early
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 phosphorylate 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 causes?
Cataracts
What does Galactosemia cause?
Cataracts, mental retardation, liver damage
What does the 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, Niemman Pick
What lysosomal diseases have a Gargoyle-face?
Gaucher’s, Hurler’s
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 MP, bone pain
What is Niemman’s Pick?
Sphingomyelinase deficiency=> zebra bodies
What is Fabry’s?
alpha-galactosidase deficiency=> corneal clouding, attacks baby’s kidneys, x-linked
What is Krabbe’s?
b-galactosidase deficiency=> globoid bodies
What is Metachromatic Leukodystrophy?
Arylsulfatase deficiency=> childhood MS
What is Hunter’s?
Iduronidase deficiency, milder form
What is Hurler’s?
Iduronnidase deficiency, worse form
What is Lesch-Nyhan?
(HGPRT deficiency) => gout, neuropathy, self-mutilation
What does white diaper crystals suggest?
Excess orotic acid
What does biotin donate methyl groups for?
Carboxylation
What does THF donate methyl group for?
Nucleotides
What does SAM donate methyl groups fpr?
All other reactions
What is the difference b/w heterochromatin and euchromatin?
Heterochromatin=tightly coiled
Euchromatin= loose (10nm fibers)
What are the purines?
A, G
What are the pyrimidines?
C,U,T
What is a silent mutation?
Changes leave te same amino acid
What is a point mutation?
Changes 1 base
What is a transition?
Changes 1 purine to another purine
What is a transversion?
Changes 1 purine to a pyrimidine
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 detect?
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 Thr Ile Met His Arg Leu Lys
What are the essential fatty acids?
Linolenic
Linoleic
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 are the smallest amino acid?
Gly
What are the ketogenic amino acids?
Lys, Leu
What are the glucogenic + ketogenic amino acids?
“PITT”
Phe, Iso, Thr, Trp
What are the glucogenic amino acids?
all the rest
What amino acids does Trypsin cut?
Lys, Arg
What amino acids does B-ME cut?
Cys, Met
What amino acids does Acid Hydrolysis denature?
Asn, Gln
What amino acids does Chymotripsin 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 Aminopeptidase cut?
Right of N terminus
What does CNBr cut?
Right of Met
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 alpha1-AT do?
Inhibits trypsin from getting loose
What is PKU?
No Phe -> Tyr (via Phe Hydroxylase): 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, Iso, Val) => aa leak out
What is Homocystinuria?
No Homocysteine -> Cys: “COLA” stones
Cystine, Ornithine, Lysine, Arginine
What is Pellagra?
Niacin deficiency
Dermatitis, Diarrhea, Dementia, Death
What is Hartnup’s?
No Trp=> Niacin + Serotonin
Presents like Pellagra
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" Vit B1=Thiamine Vit B2=Riboflavin Vit B3=Niacin Vit B4=Lipoic Acid Vit B5= Panthotenic Acid Vit B6= Pyridoxine Vit B9= Folate Viy B12=Cobalamin
What Vit A do?
Night vision, CSF production, PTH
What does Vit B1 do?
Dehydrogenases, transketolase (PPP) cofactors
What does Vit B2 do?
FAD cofactor
What does Vit B3 do?
NAD cofactor
What does Vit B4do?
Glycolisis, no known diseases
What dooes 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 HMT and MMM
What does Vit C do?
Collagen sythesis
What does Vit D do?
Mineralization of bones, teeth
What does Vit K do?
Clotting
What does Biotin do?
Carboxylation
What does Ca+2 do?
Neuronal function, atrial 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 Mg+2 do?
PTH and kinase cofactor
What does Zn +2 do?
Taste buds, hair, sperm function
What does Cr do?
Insulin fucntion
What does Mb do?
Purine breakdown (xanthine oxidase)
What does Mn do?
Glycolysis
What does Se do?
Heart function => dilated cardiomyopathy
What does Sn do?
Hair
What is Kawashiorkor?
Malabsoprtion, big belly (ascites), protein deficiency
What is Marasmus?
Starvation, skinny, calorie deficiency
What does the Pre label send stuff to?
ER
What does the Pro label send stuff to?
Golgi
Where does the mannose-6-p send stuff to?
Lysosome
Where does the N-terminal sequence send stuff to?
Mitochondria
What are the 4 types of collagen?
Strong, Slippery, Bloody, BM 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 Ehlers Danlos present?
Hyperstretchable skin
How does Marfans presents?
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 present?
Dislocated lens from top -> look down
How does Kinky hair disease present?
Hair looks like copper wire (Cu 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