USMLE Biochem Table Review
USMLE Biochem Table Review
>3')
What is the bond between two amino acids called?
peptide bond occurs in the ribosome
Where does this form?
What is the anti-codon for the start codon? CAU
What site in the rRNA does the tRNA bring the AA
to? What amount of energy is needed to translate a A-site (acceptor) 4 GTP
protein?
Primary - Amino Acids Secondary - folded AA into a-
helix or b-sheet Tertiary - 3-D the secondary
Describe the 4 levels of protein shape
structures Quarternary - multiple subunits (ex - Hb:
2a2b)
N-terminus (NH2) of newly translated protein is
Why is there "rough" ER? hydrophobic, so the rRNA will move to and attach to
the ER to complete translation within the ER
What does the golgi do to the new protein? adds sugars to the N-terminus
What AA does the golgi add N-oligosaccharides to?
N: Asparagine O: Serine and Threonine
O-oligosaccharides? (2)*
What does the golgi do if it wants to send the new Adds Mannose-6-Phosphate via phosphotransferase
protein to the lysosomes?
Dx: protein creation problem that accompanies the
signs of coarse facial features, gingival hyperplasia, Dx: I-cell disease Missing: Phosphotransferase (can't
joint immobility, growth/psychomotor retardation, send proteins to lysosomes)
cardioresp failure in rst decade. What is missing in
protein creation?
2 cells rich in RER Goblet cells of the GI Ab-secreting Plasma cells
What cell organelle is responsible for steroid Smooth ER also responsible for: detoxi cation of
synthesis? What else is it responsible for? drugs and poisons
Covalent modi cation to proteins by adding 2
negative charges to allow calcium to bind. What Gamma-carboxylation Clotting factors 2, 7, 9, 10
important function uses this method? What drug (Warfarin prevents gamma-carboxy)
prevents this?
Most prevalent AA in collagen? Two other common Glycine Proline & Lysine
AA in collagen?
Where does hydroxylation of preprocollagen occur? Proline & Lysine are hydroxylated in the ER Vitamin C
What is hydroxylated? What cofactor is needed for is required
it to occur?
What occurs to collagen after it is hydroxylated? In Glycosylated in Golgi Triple helix (Procollagen)
what part of the cell? What is formed here? Name?
Following glycosylation of collagen, what is the next
step and alteration of the structure? What is it now Procollagen is secreted from the cell. Then terminal
ends are cleaved. Then called: Tropocollagen
called?
What is needed to make the tropocollagen into
Lysyl Oxidase & Copper
brils and stabilizes this structure? (2)
(4)* Collagen synthesis de ciencies SOME: 1. Scurvy 2. Osteogenesis Imperfecta 3.
Menkes Dz 3. Ehlers-Danlos
Dx: Petechiae, ecchymoses, bleeding gums, poor Scurvy De ciency: hydroxylation of collagen
wound healing, poor bone development De ciency? de ciency secondary to lack of Vit-C
Dx: Skeletal abnormalities, multiple fractures, blue Osteogenesis Imperfecta
sclera
Dx: Hyperextensible, fragile skin, hypermobile
joints, vericose veins, arterial and intestinal Ehlers-Danlos De ciency: Lysine hydroxylase
ruptures De ciency? collagen de ciency secondary to lack of copper
To compare collagen counts of several tissues, what hydroxyproline or hydroxylysine (not Glycine--b/c it's
could be measured? prevalent in other tissues)
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Dx: 6 month infant has fractured rib; subdural Menkes disease Lysyl Oxidase in collagen
hematoma; thin, colorless, tangled hair; low serum metabolism, which requires copper
copper; developmental delay. What enzyme is
de cient?
What do pseudomonas and diphtheria toxins inhibit eEF-2: translocation factor
in eukaryotic translation?
What sequence is found at the 3' end of the tRNA? CCA then the Amino acid
What intermediate is formed by the splicing of the Lariat-shaped intermediate snRNA ("Snurp")
interons out of the mRNA transcripts? What facilitates
facilitates the splicing?
Glucose: Repressor-ON; CAP-OFF Lactose:
With the lac opeon, what is on/off if only glucose is Repressor-OFF; CAP-ON (Z, Y, A, made) Both:
present? Only Lactose? Both? When is Z, Y, A made? Repressor-OFF; CAP-OFF (glucose causes CAP off
and Lactose causes Repressor off)
What favors gene expresson--histone acetylases or histone acetylases - open hisones anything that
condenses or bulks chromatin does not favor gene
deacetylases? Why? expression
Where are enhancers located? may be upstream, downstream or within interon of
gene they control (due to looping)
When you see "zinc ngers", "leucine zippers", or Enhancers (steroid receptors are ngers, while cAMP
"helix-loop-helix", what are they talking about?
What are the ngers? response elements are zippers)
If you have multiple abnormalities with a single HOX or PAX gene
gene point mutation, where is the mutatuion?
Dx: childhood obesity and hyperphagia,
hypogonadotrophic hypogonadism, small hands and Prader-Willi syndrome Genetic Imprinting (father's
feet, mental retardation, hypotonia Type of genetic gene only)
problem?
Dx: mental retardation, continuous laughing Type of Angelman syndrome "Happy Puppet syndrome"
genetic problem? Genetic Imprinting (mother's gene only)
What does bacteria do to protect its DNA? How
does it destroy foreign DNA (bacteriophage)? it Methylates its DNA Restriction endonucleases
What do restriction endonucleases recognize in the palindromes
sequence?
Library made from nuclear DNA and is good for
seeing all sequences in the cell. Which library is Genomic Libraries cDNA Libraries
good for seeing proteins (exons) without introns?
Example of a key tissue for collagen types 1-4 Bone SCAB: I: bone, Skin II: Cartilage III: Arteries IV:
Basement membrane (4 to the oor)
What is a key feature to a Dominant genetic Dominant - every generation is affected (not in
problem in pedigree (vs. recessive)? recessive)
What is a key feature to Autosomal genetic problem Autosomal - Male-to-male inheritance (x-linked has
in pedigree (vs. X-linked)? no male-to-male)
Dx: Lateral displacement of the innercorner of the
eye; Pigment abnormalities; congenital deafness; Klein-Waardenburg Syndrome HOX gene
limb abnormalities What gene is the problem? abnormality
If cystic brosis patient were to be treated with
gene therapy, which type of cells should be targeted Epithelial cells
by the host cells?
FH-MAN Familial Hypercholesterolemia;
name 5 Autosomal Dominant diseases Huntingtons; Marfans; Acute Intermittent Porphyria;
NF-1
What is usually the common problem in the Structural protein damage
Autosomal Dominant diseases?
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name 4 common Autosomal Recessive diseases SCriPT Sickle cell; Cystic Fibrosis; PKU; Tay-Sachs
What type of genetic diseases affect energy Mitochondrial Diseases: Leber's optic neuropathy;
production? (Name 3) MELAS; MML
name 4 X-linked recessive common diseases Duchennes muscular dystrophy; Lesh-Nylan; G6PD;
Hemophilia A & B
Paternal relationships b/t man and infant can best
be determined by DNA ngerprinting. What about Tandem repeats
the DNA is analyzed?
2 amino acids that are the precursors for Phenylalanine Tyrosine
catecholamines
What NT does tryptophan form? What vitamin? Serotonin Niacin
3 branched-chained amino acids found in Maple I Love Vermont: Isoleucine; Leucine; Valine
Syrup Urine disease?
name the (6)* nonpolar, non-aromatic hydrophobic VIP GAL: Valine; Isoleucine; Proline Glycine; Alanine;
amino acids Leucine
Only amino acid with a phenol group? Tyrosine
name the 3 aromatic amino acids Phenylalanine Tyrosine Tryptophan
the amino acid that acts as a methyl donor methionine (SAM)
name 3 amino acid that are basic What is the pK of HLA: Histidine (6.5) Lysine (10) Arginine (13)
the R groups?
name the 2 amino acids that are acidic What is the
pK of the R groups? Glutamate (4) Aspartate (4)
What are the 2 pKs that every amino acid has? pk (COO-) = 2 pk (NH2) = 9
What is the net charge on the amino acid if the pH is
less then the pI? positive
What is the pI? (equation) average of the pKs: (2+9)/2 = 5.5 for normal AA
[average the 2 closest numbers for charged AA]
Main extracellular buffer bicarbonate
10 essential amino acids PVT TIM HALL: Phe-Val-Thr Trp-Ile-Met His-Arg-
Leu-Lys
What 2 essential amino acids are required during
periods of growth? Histidine Arginine
What is the sign of delta G in a spontaneous negative
reaction?
Affect on Km & Vmax: 1) Competitive Inhibitor 2) Competitive: Km= Increase; Vmax= no change Non-
Non-competative Inh competitive: Km= no change; Vmax= Decrease
what does the x and y axis measure on Lineweaver- x-axis: -(1/Km) y-axis: (1/Vmax)
burke plots?
How does af nity relate to Km? low Km = high Af nity
amino acid precursor to GABA Glutamic Acid
amino acid precursor to Heme Glycine
the amino acid that is the precursor for: Creatine
Urea Nitric Oxide Arginine
adds a phosphate to a substance Kinase
2 serine kinases cAMP cGMP
which receptor type has no g-protein and results in
smooth muscle relaxation? Second messenger? cGMP Protein kinase G
What phosphorylates in the insulin/glucagon world? Glucagon - phosphorylates (needs ATP) Insulin - de-
Which needs ATP? phosphorylates
removes a phosphate from a substance Phosphatase
What bug affects ADP-ribosylation at Gs stimulates Gs- Cholera (stimulates the stimulator)
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receptors? What bug affects it at Gi? What do they inhibits Gi- Pertussis (inhibits the inhibitor) Both
both cause in the cell? cause: Increase in cAMP
What two Vitamins can be used in dehydrogenase
reactions? Ribo avin (B2) Niacin (B3)
Vitamin that makes CoA? Pantothenic Acid (B5)
What (4)* things does the liver produce in a well-fed G-TAB: GLYCOGEN Triglyerides ATP Bile (from
state? cholesterol)
What is the rst step in a well-fed state for the
following tissues: liver, RBC, brain, muscle, fat? GLYCOLYSIS
2 actions of RBC in a well-fed and fasting state? 1) glycolysis (for ATP) 2) Lactate production
What tissues use insulin receptors: GLUT 1 and 3? RBC Brain
What tissues use receptor: GLUT 2? Liver
What tissues use receptor: GLUT 4? Muscle Adipose
Once there is enough energy in liver during well-fed Glucose & Acetyl-CoA Glucose - added together to
state, what 2 substances build-up? What is done be stored as glycogen Acetyl-CoA - added together to
with them? become fatty acids (then transported to adipose via
VLDL)
What does adipose do during well-fed states? takes in glucose and fatty acids to make ATP and fat
What (4)* substances enter the liver during a well- Goes to Liver And Grows: Glucose - food (blood)
fed state? Where is each from? Lactate - RBC Amino Acids - food (blood) Glycerol -
Chylomicrons (food)
What does the liver give to tissues during fasting
states? To what tissues? Glucose - RBC & Brain Ketone bodies - muscle
What does muscle get during the fasting state? (2) Ketone bodies - liver (once enough ATP is made and
From where? Aceytl-CoA builds-up) Fatty acids - Adipose (carried
on albumin)
What does the muscle provide to the liver during Amino Acids 1. to make Pyruvate (then glucose) 2. for
fasting states? For what? (2) Urea cycle
2 sources (& preliminary substances) to make RBC - lactate muscle - Alanine
pyruvate b/t meals for the liver to make glucose
What 2 ways can the GLUT-4 receptor move to the
1) Increased Insulin 2) Exercise
surface of muscle?
What are the 2 anaerobic steps of substrate level 1,3BPG --> 3PG (via Phosphoglycerate kinase) PEP --
phosphorylation in glycolysis? > Pyruvate (via Pyruvate kinase)
What are two essential items needed to make ATP 1) Oxygen 2) Mitochondria (for ETC)
from NADH?
First and third step: Glucose --> G6P (via
What 2 steps of glycolysis use ATP? Hexokinase/Glucokinase) F6P --> F-1,6-BP (via PFK-
1)
What is the rate-limiting step of glycolysis? Net PFK-1 2 ATP (net)
ATP?
How does the RBC replenish NADH? With what Pyruvate --> Lactate (Enzyme: Lactate DH) changes
essential enzyme? If enzyme was not there, what NAD+ --> NADH for glycolysis (lactate goes to liver)
would occur? If Lactate DH not there, RBC would die
What enzyme of glycolysis is only in the liver and is Glucokinase Low af nity = High Km = high Vmax
induced by insulin? Level of af nity of this enzyme?
Insulin (dephos) stimulates PFK-2 for: F6P --> F-2,6-
How is PFK-1 controlled? Which form is active BP to make PFK-1 Glucagon (phosphorylates)
(dephosphorylated or phosphorylated)?
inhibits PFK-2
What (3)* metabolic events occur in the Fatty acid oxidation Acetyl-CoA production TCA
mitochondria? cycle
What (5)* metabolic events occur in the cytoplasm? Glucose Found Here Protects Steroids: Glycolysis
Fatty acid synthesis HMP shunt Protein synthesis
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What enzyme in Purine synthesis is the rate- PRPP Amidotransferase Drugs ("Purin"):
limiting step? What (2) drugs inhibit this step? AlloPURINol (Gout); 6-MercaptoPURINe (cancer)
What intermediate in purine synthesis is different
from guanine and adenine by one amino group and Hypoxanthine
is also an intermediate in the breakdown of purines?
Lack of the salvage pathway of purines leads to
LNS = Lacks Nucleotide Salvage: Lesch-Nyhan
what syndrome? What is the missing enzyme? What
syndrome HGPRTase Sign: Self-mutilation
is an unusual sign of this syndrome?
Adenosine Deaminase de ciency causes a lack of
what product? Inosine
Dx: 48-yo man w/ recessive condition is at high risk
for deep vein thrombosis and has had replacement Homocysteinuria Enzyme: Cystathionine Synthase
of ectopic lenses; no evidence of anemia. Enzyme AA: Methionine
that is de cient? What AA would be in excess?
Dx: Man has dark pigment of cartilage and arthritis, Alkaptouria Enzyme: Homogentisate Oxidase [AL
and dark urine What enzyme is de cient? KAPT a HOMe]
How do you treat Cystinuria? Alkalinize the urine w: Acetazolamide
Precursor to Ketone body formation HMG-CoA
Try HUNTING for MY FRIED X (eggs): Huntington's;
(4)* genetic Dz w/ Trinucleotide repeats
Myotonic Dystrophy; Friedreich's Ataxia; Fragile X
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