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Biochemistry

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Answer 50 questions in 15 minutes.
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Match each statement with the correct term.
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1. What two rxns in in glycolysis require ATP
NADH - NADPH - FADH2
Binds to LDL receptor - mediates VLDL secretion
CAG
Glucose to G-6P - hexokinase and fructose -6P to fructose 1 -6 BP phosphofructokinase -1

2. What is the energy source in the fed state right after a meal
Medial dorsal nucleus of thalamus - mamillary bodies
Glycolysis and aerobic respiration
Excess NADH shunts oxaloacetate to malate (backwards) stalling the TCA and shunting glucose and FFA to ketone body production
Tryptophan excretion in urine and dec absorption from the gut leading to pellagra

3. What two amino acids are required druing periods of growth and why
Hepatic glycogenolysis - adipose tissue release of FFA - muslce and liver FFA - hepatic gluconeogenesis from peripheral tissue lactate and alanine and from adipose tissue glycerol and proprionyl - coA (odd chain FFA)
ATP and methionine
Arg and his inc in histones Which bind negatively charged DNA
Activates LCAT

4. What lab procedure is used to amplify desired fragment of DNA and What are the 3 steps
PCR - denaturation - annealing - elongation
Convulsions - hyperirritability - peripheral neuropathy - deficiency inducible by INH and oral contraceptives - sideroblastic anemais
Prokaryotic only - degrades RNA primer and fills in the gap with DNA
Inc melt - dec fluidity

5. What defects characterize velocardiofacial syndrome
FMR1 gene - methylation - associated with chromosomal breakage
One
Eu - methionine - pro - formyl - methionine
Palate - facial and cardiac defects

6. What are the functinos of vitamin A
Antioxidant - constituent of visual pigment - essential for nl differntiation of epithelial cells into specialized tissue (pancreatic cells - mucus secreting cells) used to treat measles
Macrocytic - megaloblastic anemia - no neurologic sx - most common vit def in the US - seen in EtOH and pregs
L form
Intermediate filaments

7. What does biotin def cause
Dermatitis - alopecia - enteritis
Protective against malaria
B(1 - 2 - 3 - 5 - 6 - 12) C - biotin and folate (B12 and folate are stored)
Polyneuritis - symmetrical muscle wasting

8. What causes Marfan syndrome
OTC has hyperammonemia - orotic aciduira does not
Defect in fibrillin
Biotin
Acetoacetate and beta hydroxybutyrate

9. What residues are hydroxylated in step 2 of collagen synthesis - and What does this require
Proline and lysine - vit C
HMG- CoA (HMG- CoA to mevalonate
Liver and beta cells of pancreas - low affinity - high Km - high capacity - high Vmax - induced by insulin
N to C

10. What is the activated carrier for 1 carbon units
THFs
4 under the floor
AD absent of dec LDL receptors causes accelerated atherosclerosis - achilles xanthomas and corneal arcus - increase LDL and elecated cholesterol
Evident in first few days of life - can present last onest - excess carbamoyl phosphate converted to orotic acid - orotic acid in blood and urine - dec BUN and symptoms of hyerpammonemia

11. What is imprinting and give an example

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12. Which carbon bears the triphosphate and the energy source for bond formation

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13. What is the treatment for dec affinity of cystathionine synthase for pyroxidal phosphate
Inc NADH/NAD ratio in liver - causing diversion of pyruvate to lactate and OAA to malate - inhibiting gluconeogenesis and stimulating fatty acid synthesis. Leads to hypoglycemia and hepatic fatty liver change seen in chronic EtOH users
Enhancers
Common metabolites like pyruvate and acetyl CoA and excess NH4+ converted to urea and exreted
Inc vit B6

14. How does ouabain work
Failure to track objects or develop a social smile
Autosomal recessive diseases
Liver - ovaries - seminal vesicles
Inhibits the Na/K pump by binding the K side

15. What does pancreatic lipase do
Terminal regions - tropocollagen
Inhibits the Na/K pump by binding the K side
Degredation of dietary TG in small intestine
Depletes alpha - ketoglutarate leading to inhibition of TCA cycle - tremor slurring of speech - somnolence - vomiting - cerebral edema - blurring of vision

16. What is the physiologic role of dystrophin
2 rings
N- acteylcysteine - cleave disulfide bonds within mucus glycoproteins
Anchor muscle fibers - primarily in skeletal and cardiac muscle
Wobble

17. What are possilbe presentation for galactokinase def
Two
Failure to track objects or develop a social smile
Hypercalciuria - loss of appetite - stupor - seen in sarcoidosis from activation fo vit D by epithelioid macrophage
Inc vit B6

18. Why enzyme breaks down elastin and what enzyme inhibits it
Glutamine PRPP amidotransferase
Liver and beta cells of pancreas - low affinity - high Km - high capacity - high Vmax - induced by insulin
Seals.
Elastase - inhibited by alpha1 antitrypsin

19. What does apoE do
Neural tube
Scrutinizes - hydrolyzes the bond
Mediates extra remnant take up
1 ring

20. What is sorbitol - how and why is it made
Liver - ovaries - seminal vesicles
Alcohol version of glucose - can trap glucose in cell - aldose reductase
Terminal regions - tropocollagen
ATP and alanine

21. What sugar is sorbitol converted to and via what enzyme - and What can happen in cells lacking this enzyme
RER - translation of alpha chains - usually Gly-X-Y polypeptide (preprocollagen)
Fructose via sorbitol dehydrogenase - inc sorbitol leading to osmotic damage as in cataracts - retinopathy - peripheral neuropathy as in diabetes
Polycystic liver disease - berry aneurysm - mitral valve prolapse
Inc insulin - dec cAMP - dec PKA

22. How is orotic aciduria inherited
AR
32 - malate aspartate shuttle
Alanine
Lariat shape in order and remove intron precisely and join 2 exons

23. What is DNA cloning and How do you do it?
Acetyl - CoA carboxylase (ACC)
Production of a recombinant DNA molecule that is self perpetuating - plasmids - selection - restriction enzyme cleavage - tissue mRNA with reverse transcriptase to make cDNA
Abnormal protein folding - degradation before reaching cell surface
Many generations - both male and female affected - often pleiotropic - can present clinically after puberty

24. Wgat substances directly inhibit mitochondrial ATPase - causing an inc in proton gradient - no ATP because pump is stopped
Exons
African Americans and Asians
Breaks down acyl - coa to acetyl coa groups in mito
Oligomycin

25. In a marathon Where does energy come from
Acetoacetate and beta hydroxybutyrate
Glycogen and FFA oxidation; glucose conserved for final sprinting
Mental retardation - growth retardation - seizures - fair skin - eczema - musty body odor
Post to neg

26. What is the breakdown product of epi
HMG- CoA reductase
Metanephrine
Fibrofatty replacement of muscle - cardiac myopathy
Von gierkes - glucose 6 phosphatase

27. What does the start codon code for in eukaryotes and prokaryotes
Schwann cells - lens - retina - kidneys
Eu - methionine - pro - formyl - methionine
Mediates chylomicron secretion
Adds an inorganic phosphate onto substrate without using ATP

28. Where is EtOH dehydrogenase located
Inc orotic acid in urine - megaloblastic anemia (does not correct with B12 or folate) - failure to thrive
Cytosol
Prokaryotic only - degrades RNA primer and fills in the gap with DNA
TTP

29. What occurs to oxaloacetate in starvation and DKA
Alpha1 antitrypsin
Depleted for gluconeogenesis staling the TCA cycle and shunting glucose and FFA to production of ketone bodies
Dermatitis - enteritis - alopecia - adrenal insuff
Hypoglycemia

30. What tissues have both enzymes of sorbitol metabolism
Hypercalciuria - loss of appetite - stupor - seen in sarcoidosis from activation fo vit D by epithelioid macrophage
Purines= A - G pyrimidine = C - T (U)
Liver - ovaries - seminal vesicles
Pyrophosphate (B1 - thiamine - TPP) FAD (B2 - riboflavin) - NAD (B3 - niacin) - CoA (B5 pantothenate) - lipoic acid

31. Mild Hurlurs + aggressive behavior no corneal clouding
Hunters - iduronate sulfatase - heparan sulfate/dermatan sulfate - XR
HMG- CoA synthase
Foliage - small reserve in liver - eat green leaves
Leu - lys

32. What does degenerate/rundant genetic code refer to...
Retardation - self mutilation - aggression - hyperuricemia - gout - choreoathetosis
Glucose -6 phosphate
More than 1 codon may code for the same amino acid
Early in life - AR - inability to synthesize lipoproteins due to def in apoB100 and B48 - intestinal biopsy shows accumulation within enterocytes due to inability to export absorbed lipid as chylomicrons - failure to thrive - steatorrhea - acanthocyt

33. cytoskeletal elements associated with vimenentin - desmin - cytokeratin - glial fibrillary acid protiens (GFAP) - neurofilaments
Tyrosine
Intermediate filaments
Glycine
Remain in G0 - regenerate from stem cells - neurons - skeletal/cardiac muscle - RBCs

34. What enzyme converts adenine to AMP
Scurvy - swollen gums - bruising - hemarthrosis - anemia - poor wound healing - weakened immune response
APRT + PRPP
Lactate
Stored and undergoes glycogenolysis to maintain blood sugar at appropriate levels

35. What is trimming
Antioxidant - protects RBCs and membrances from free radical damage
Lack of tyrosinase (no melanin) AR ordefective tyrosine transporters (less tyrosine) - can results in lack of migration of NC cells
Abnormal protein folding - degradation before reaching cell surface
Removal of N or C termal propeptides from zymogens to generate mature proteins

36. What is the amino acid precuros for niacin and serotonin/melatonin
Tryptophan
Schwann cells - lens - retina - kidneys
Elastase - inhibited by alpha1 antitrypsin
Inc Cl - in sweat

37. What is the Name and fxn of vit B12
B100
Oxidized hemoglobin precipiated within RBCs
F16BP
Cobalamin - cofactor of homocysteine and methyltransferase - methylmalonyl CoA mutase

38. Eukaryotic and prokaryotic DNA synthesis is blank and involves both blank and blank strands
Transmitted only through mother - all offspring of affected females may show signs of disease
Inhibits the Na/K pump by binding the K side
Semiconservative - continuous and discontinuous strands (okazaki fragments)
Consesus sequenec of base pairs

39. What does SAM do
Cri du chat
Neural tube
Alpha and beta tubulin - dimers have two GTP bound
Transfers methyl units

40. Which step in the de novo purine and pyrimidine synthesis pathway requires aspartate - glycine - glutamine and THF
Polycystic liver disease - berry aneurysm - mitral valve prolapse
Von hippel lindau - 3
Ribose 5- P to PRPP
Aldolase B - AR - fructose 1P accumulates causing a dec in availabel phosphate which inhibts glycogenolysis and gluconeogenesis leading to hypoglycemia - cirrhosis - jaundice and vomiting

41. cytoskeletal elements in micorvilli - muscle contracting fibers - cytokenesis - adherens jxns
CGG
Actin and myosin
Glycolysis - fatty acid synthesis - HMP shunt - protein synthesis (RER) - steroid synthesis (SER)
Regulatory proteins that control cell cycle events; phase specific; activate CDKs

42. Synthesis of vit B3 requires what other vitamin
Liver and leafy veggies
B6
Targets the proteins for lysosome
Mental retardation - macro - orchidism - long face with large jaw - large everted ears - autism - and mitral valve prolapse

43. What is the TX for CF and What does it do
Transfers methyl units
Nature and severity of phenotype vary from 1 individual to another - NF type 1
Hereditary hemorrhagic telangiectasia (Osler - Weber - Rendu syndrome)
N- acteylcysteine - cleave disulfide bonds within mucus glycoproteins

44. What is the RDE of de novo pyrimidine synthesis
Anabolic processes as a supply of reducing equivalents
AMP - fructose 2 -6 BP
Transfers methyl units
Carbomoyl phosphate synthetase II

45. Type II collagen
Riboflavin - cofactor in oxidation and reduction (FAD and FMN are derived from riboflavin)
Cartilage - hyaline - vitreous body - nucleus pulposus
Histidine
Tropoelastin with fibrillin scafolding

46. Which direction does dynein go
Carbomoyl phosphate synthetase II
Post to neg
Methionine encode by only 1 codon (AUG)
Transfers cholesterol from mature HDL to VLDL - IDL and LDL (cholesterol ester transfer protein)

47. Which anti breast cancer drugs work on micortubules
Targets the proteins for lysosome
Males are infertile due to bilateral absence of vas deferens
Polycystic liver disease - berry aneurysm - mitral valve prolapse
Paclitaxel

48. Where is vit A found in the diet
Liver and leafy veggies
Inc Cl - in sweat
Ubiquitous - low Km - high affinity - low capacity low Vmax - uninduced by insulin
More than 1 codon may code for the same amino acid

49. What does the golgi apparatus do on asparagine
SAM
Modifies N- oligosaccharides
Rickets in kids - bending bones - osteomalacia in adults - hypocalcemia tetany - breast milk has dec vit D (supplement in dark skinned patients)
Exons

50. What is the most common urea cycle disorder and What is the mode of inheritance?
Niacin - constituent of NAD and NADP - derived from tryptophan
Ornithin transcarbamoylase def - x linked recesssive - other urea cycle enzymes defs are autosommal recessive
Supply sufficient glucose to brain and RBCs and to preserve protein
Defect in fibrillin