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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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This is a study tool. The 3 wrong answers for each question are randomly chosen from answers to other questions. So, you might find at times the answers obvious, but you will see it re-enforces your understanding as you take the test each time.

1. Which are the acidic amino acids
Aberrant 3rd and 4th branchial arches causing 22q11 deletion - cleft palate - abnl facies - thymic dysplasia - cardiac defects - hypocalcemia secondary to parathyroid aplasia
Asp and Glu
Liver - also in kidney and gut epithelium
Glycogenolysis to form glucose

2. What does apoB48 do
Schilling test
Pantothenate - essential component of CoA - a cofactor for acyl transfers and fatty acid synthase
Oral uridine administration
Mediates chylomicron secretion

3. How do glucagon/epi lead to glycogenolysis
Microarrays
Supply sufficient glucose to brain and RBCs and to preserve protein
Stop codon is recognized by release factor - and completed protein is released from ribosome
Adenylyl cycle - inc cAMP - inc PKA - glycogen phosphorylase kinase activated - glycogen phosphoylase active - glycolysis

4. What is the breakdown product of dopamine
Confusion - opthalmoplegia - ataxia - confabulation - personality changes and memory loss
Liver and beta cells of pancreas - low affinity - high Km - high capacity - high Vmax - induced by insulin
HVA
Glycogen phosphorylase

5. Who typically has lactase def
Same as sprint + OXPHOS
Dermatitis - alopecia - enteritis
African Americans and Asians
Dec AFP - inc betahCGH - dec estradiol - inc inhibin - inc nuchal translucency on US

6. In mismatch repair - which strand is recognized and In what hereditary syndrome is this defective
Cori's - debranching enzyme alpha 1 -6 glucosidase - gluconeogenesis intact
After citruline
Unmethylated - newly synthesized - HNPCC
Antigen - antibody reactivity - can look for antigen or antibody in patients blood

7. 1 g of protein or cabrohydrate = ?kcal
Marasmus - muscle wasting
Four
PFK - rate limiting enzyme
Prokaryotic only - elongates leading strand by adding deoxnucTIDE to the 3' end. Elongates lagging strand until it reaches primer of preceeding fragment. 3' to 5' exonuclease activity 'proofreads' each added nucTIDE

8. Why can't even chain fatty acids produce new glucose
Autosomal recessive diseases
Neither of 2 alleles is dominant - blood groups
1 ring
They yield only acetyl - CoA equivalents

9. What happens with wet beriberi
Meiotic nondisjunction of homologous chromosomes (associated with advanced maternal age) leading to trisomy 21
High output cardiac failure - dilated cardiomyopathy - edema
Fibroblast
RNA

10. What does Citrate Is Krebs starting substrate for making oxaloacetate
CO2 + NH4 needs carbamoyl phosphate synthase I - in the mitochondria
Citrate - isocitrate - alpha ketoglutarate - succinyl - CoA - succinate - fumarate - malate - oxaleoacetate
Cofactor for LPL
Hereditary defect of renal tubular amino acid transporter for cystein - ornithine - lysine and arginine in PCT

11. What does cytokeratin stain for
ATP and methionine
Epithelial cells
Hepatic glycogenolysis >> hepatic gluconeogenesis > adipose release of FFA
III - joint dislocation - anuerysms - organ rupture

12. Which aspect of the spliceosome do patients with lupus make antibodies against
Oxidized hemoglobin precipiated within RBCs
Von Gierke's - Pompe - Cori - McArdle
SnRNPs
Adenosine to inosine

13. Which antifungal drugs work on microtubules
Lungs - large arteries - elastic ligaments - vocal cords - ligamenta flava - stretch.
Dopa decarboxylase
Griseofulvin
Inc Cl - in sweat

14. What form of amino acids are found in proteins
Scrutinizes - hydrolyzes the bond
L form
Lack of tyrosinase (no melanin) AR ordefective tyrosine transporters (less tyrosine) - can results in lack of migration of NC cells
Met - val - arg his

15. What clotting factors require vit K
Covalent cross - linking by lysyl oxidase to make collagen fibrils
HMG- CoA (HMG- CoA to mevalonate
II - VII - IX - X (1972) protein C and S
Neurofibromatosis type 1 (von Recklinghausens disease)

16. Why is albinism inheritnace varialbe due to...
Liver - ovaries - seminal vesicles
Locus heterogeneity - ocular albinism is x- linked recessive
Liver - also in kidney and gut epithelium
AMP - fructose 2 -6 BP

17. How does ouabain work
2 -4 DNP - aspirin
Inhibits the Na/K pump by binding the K side
Tyrosine
Inhibit DNA gyrase specific for prokaryotic topoisomerase

18. What does inc phenylalanine lead to...
NADPH
Phenylketones in urine
Coarse facial features - clouded corneas - restricted joint movements - high plasma levels of lysosomal enzymes - often fatal in childhood
Medial dorsal nucleus of thalamus - mamillary bodies

19. What can excess vit B3 cause
Exercise: inc NAD/NADH - inc ADP - inc Ca
Scrutinizes - hydrolyzes the bond
Facial flushing
NAD+

20. What happens in termination of proteins synthesis
FISH
Stop codon is recognized by release factor - and completed protein is released from ribosome
Cytosol - F 1 -6 BP to fructose 6 Phosphate
Tryptophan excretion in urine and dec absorption from the gut leading to pellagra

21. The pyruvate dehydorgenase complex serves In what reaction: reactants
Pyruvate - NAD+ - CoA
Backup of substrate (pyruvate and alanine) resulting in lactic acidosis - congenital or acquired from thiamine def in EtOH - neuro defects
Lysine and arginine
Phenylalanine hydroxylase

22. What is made fir rNucleotides or deoxyrNucleotides and what enzyme is necessary to convert one to the next
Ribos first then deoxyribos with ribonucleotide reductase
Vincritsine/vinblastine
Grows slowly - collapses quickly
Arginine

23. How many rings do pyrimidines have
Histidine
1 ring
GAA
B12 and folate

24. Type IV BM
Von gierkes - glucose 6 phosphatase
Oxidative and nonoxidative - no ATP produced or used
Fatty acid oxidation - acetyl - Coa production - TCA cycle - OXPHOS
4 under the floor

25. How do stable (quiescent) cells grow and regenerate and What are examples
Hartnup disease - dec tryptophan absoprtion - malignant carcinoid syndrome (inc tryptopha metabolism) and INH (dec vit B6)
L form
Glutamate
Enter G1 from G0 when stimulated - hepatocytes and lymphocytes

26. Name 5 drugs that interfere with nucleotide synthesis
4 under the floor
Hydroxyurea - inhibits ribonucleotide reductase - 6 MP - blocks de novo purine synthesis - 5 FU inhibits thymidilate synthase - MTX - inhibits DHFR - Trimethroprin inhibits bacterial DHFR
Von Gierke's - Pompe - Cori - McArdle
Histidine

27. What does the deletion of the dystrophin gene lead to...
Accelearted muscle breakdown
Oxidized hemoglobin precipiated within RBCs
TTP
Phenylketones in urine

28. What shuttle is used in fatty acid degredation and What does it move and From where to where
Provide a source of NADPH from an abundantly available glucose 6P - create ribose for nucleotide synthesis and glycolytic intermediates
P+q = 1
Activates LCAT
Carnitine shuttle - acyl - coa from cyto to mito

29. What is incomplete penetrence and give an example
Microtuble polymerization defect causing a dec in phagocytosis results in recurrent pyogenic infections - partial albinism - and peripheral neuropathy
Karyotyping
Not all individuals with a mutant genotype show the mutant phenotype
Autosomal recessive diseases

30. What rxn does propionyl - CoA carboxylase catalyze
B6
FAP
Alpha 1 -4 glucosidase
Propionyl - CoA to methylmalonyl - CoA (3C to 4C)

31. What is axonemal dynein
Inhibits EtOH dehydrogenase and is an antidote for methanol and ethylene glycol poisoning
Alpha 1 -4 glucosidase
Transmitted through both parents - affected mother may have affected children - affected father will have affected children
ATPase that links peripheral 9 doublets and causes bending of cilium by differential sliding of doublets

32. What is the complication of cystinuria
Supply sufficient glucose to brain and RBCs and to preserve protein
1 gene had >1 effect on an individuals phenotype - PKU causes many seemingly unrelated symptoms ranging from mental retardation to hair/skin changes
ADPKD
Cystine kidney stones - cystine staghorn calculi - cystine is made of two cysteines connected by disulfide bond

33. nucleotide repeat for fredreich's ataxia
GAA
Peroxide
Microarrays
Anabolic processes as a supply of reducing equivalents

34. What does a pyruvate dehydrogenase deficiency lead to and What are the findings
Backup of substrate (pyruvate and alanine) resulting in lactic acidosis - congenital or acquired from thiamine def in EtOH - neuro defects
2 rings
Rickets in kids - bending bones - osteomalacia in adults - hypocalcemia tetany - breast milk has dec vit D (supplement in dark skinned patients)
Marfans

35. Synthesis of vit B3 requires what other vitamin
B6
The triphosphate bond
Excess NADH shunts oxaloacetate to malate (backwards) stalling the TCA and shunting glucose and FFA to ketone body production
Inhibit DNA gyrase specific for prokaryotic topoisomerase

36. What does a southern blot use as its sample
Type II
Inhibits acetaldehyde dehydrogenase and contributes to hangover symptoms
DNA
Schwann cells - lens - retina - kidneys

37. Which phase of the HMP shunt is reversible and Which is irreversible
Wrinkles and acne
1- capping on 5' (7- methyguanosine) 2 - polyadenylation on 3' end 3 - splicing out of introns
Oxidative is irreversible
Wobble

38. What does beta oxidation do and Where does it occur
Inc CPK and muscle biopsy
Phenylalanine hydroxylase - tetrahydrobiopterin cofactor
Binds 50S - blocking translocation
Breaks down acyl - coa to acetyl coa groups in mito

39. inc glycogen in muscle but can't break it down - painful muscle cramps - myglobinuria with strenuous exercise - dz and enzyme

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40. Where is glucose 6 phosphatase found and What does it do
P+q = 1
FAP
Tryosine hydroxylase
In ER - glucose 6- P to glucose

41. Type III collagen
Hepatic glycogenolysis >> hepatic gluconeogenesis > adipose release of FFA
Deamination
Reticulin - skin - blood vessels - uterus - fetal tissue - granulation tissue
AD absent of dec LDL receptors causes accelerated atherosclerosis - achilles xanthomas and corneal arcus - increase LDL and elecated cholesterol

42. What are the glucogenic essential amino acids
Propionyl - CoA to methylmalonyl - CoA (3C to 4C)
30 - glycerol -3- phosphate shuttle
Passed to glutamate - then to alanine enters blood - enters liver - coverted to pyruvate and transfers ammonium back to glutamate which gives it to the urea cycle
Met - val - arg his

43. What enzyme converts adenine to AMP
OTC has hyperammonemia - orotic aciduira does not
Cori's - debranching enzyme alpha 1 -6 glucosidase - gluconeogenesis intact
Degredation of TG circulating in chylomicrons and VLDLs
APRT + PRPP

44. What is the result of vit B5 def
AD absent of dec LDL receptors causes accelerated atherosclerosis - achilles xanthomas and corneal arcus - increase LDL and elecated cholesterol
Glucose 6 phosphate dehydrogenase (G6PD)
Oxalacetate
Convulsions - hyperirritability - peripheral neuropathy - deficiency inducible by INH and oral contraceptives - sideroblastic anemais

45. What 3 steps in RNA processing occur after transcription

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46. What are the fetal screening measures for Down
Citrate - isocitrate - alpha ketoglutarate - succinyl - CoA - succinate - fumarate - malate - oxaleoacetate
Phosphenolpyruvate to pyruvate catalyzed by pyruvate kinase
Dec AFP - inc betahCGH - dec estradiol - inc inhibin - inc nuchal translucency on US
Actin and myosin

47. Pts with albinism are at inc risk For what cancer
Liver - ovaries - seminal vesicles
Skin
Comlex II
Glossitis - severe = pellagra - diarrhea - dermatitis - dementia

48. ADPKD is associated with What additional conditions
Polycystic liver disease - berry aneurysm - mitral valve prolapse
Receptor tyrosine kinases - protein phosphatase - takes phosphate off glycogen phosphorylase kinase inactivating it
Arg - lys - his - arg is most basic - has has no charge at body pH
Tryptophan

49. What does the passage of electrons result in that when coupled to OXPHOS drives the production of ATP
Absence of galactose 1 phosphate uridyltransferase - accumulation of toxic substances leads to failure to thrive - jaundice - hepatomegaly - infantile cataracts - mental retardation
Result from phagocytic removal of heinz bodies my macs - G6PD def
Achondroplasia
Proton gradient

50. What is locus heterogeneity and give an example
Delayed wound healing - hypogonadism - dec in adult hair - dysguesia - anosmia - may predispose to EtOH cirrhosis
Mutations at different loci can produce the same phenotype - marfans - MEN 2B - homocystinuria; all cause marfinoid habitus - also albinism
Aminoacyl - tRNA binds A site - ribosomal rRNA catalyzes peptide bond formation - transfers growing peptide to AA in A site - ribosome advances 3 nucTIDE toward 3' end of RNA - moving peptidyl RNA to P side
Fructose via sorbitol dehydrogenase - inc sorbitol leading to osmotic damage as in cataracts - retinopathy - peripheral neuropathy as in diabetes