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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 happens on the nonoxidative arm of the HMP shunt and What is the key enzyme and cofactor
Ribulose 5P to ribose 5P - G3P and F6P - transketolase and B1
Oxidizes substrate
NAD+
Mediates chylomicron secretion

2. cytoskeletal elements associated with cilia - flagella - mitotic spindle - neurones and centrioles
Microtubules
Grows slowly - collapses quickly
Skin
Polyneuritis - symmetrical muscle wasting

3. How many ATP are produced by anearobic glycolysis per molecule of glucose
Fed
Flank pain - hematuria - HTN - progressive renal failure
Two
Orotic acid to UMP

4. What regulates whether FBPase -2 or PFK-2 is active
Inc permeability of membrane causing a dec in proton gradient and inc in O2 consumption - ATP synthesis stops - but electron transport continues to produce heat
Vit K antagonist
Protein kinase A
Inc intake of ketogenic nutrients - high in fact content or inc lysine or leucine

5. bilateral acoustic schwannomas - juvenile cataracts
III - joint dislocation - anuerysms - organ rupture
NF2 on chromosome 22
Pyruvate carboxylase - PEP carboxykinase - fructose 1 -6 biphosphatase - glucose 6 phosphatase
HMG- CoA synthase

6. Wgat substances directly inhibit mitochondrial ATPase - causing an inc in proton gradient - no ATP because pump is stopped
NAD+
Alpha and beta tubulin - dimers have two GTP bound
Oligomycin
Semiconservative - continuous and discontinuous strands (okazaki fragments)

7. What metabolic rxns occur in both the cytoplasm and mitochondria
Essential fructosuria - fructokinase AR
Heme synthesis - urea cycle and gluconeogenesis - HUGs take two
Glucose -6 phosphate
Phosphorylation - glycosylation - hydroxylation

8. What are covalent alterations
ATP hyrdolysis couple to energetically unfavorable rxns
Phosphorylation - glycosylation - hydroxylation
AR disorder characterized by defective neutral amino acid transporte on renal and intestinal epith cells
B12 and folate

9. deletion of VHL gene on chromosome 3 leading to hemangioblastomas of retina/cerebellum/medulla bilateral RCC - dz and overexpression product
Promotor - TATA box - and CAAT box - AT rich
Von hippel lindau - 3
Base + ribose
Malabsorption (sprue - enteritis) lack of intrinsic factor from pernicious anemia - gastric bypass surgery or absence of terminal ileum as in Crohns

10. What is the RDE of TCA cycle
Locus heterogeneity - ocular albinism is x- linked recessive
Carnitine shuttle - acyl - coa from cyto to mito
Isocitrate dehydrogenase
Exercise: inc NAD/NADH - inc ADP - inc Ca

11. What is the activated carrier for 1 carbon units
Marasmus - muscle wasting
THFs
HMG- CoA reductase
RER - translation of alpha chains - usually Gly-X-Y polypeptide (preprocollagen)

12. When does aspartate enter the urea cycle
Wrinkles and acne
Von Gierke's - Pompe - Cori - McArdle
Flagella - cilia - mitotic spindles
After citruline

13. What enzyme converts phenylalanine to tyrosin
Supply sufficient glucose to brain and RBCs and to preserve protein
RRNA
Phenylalanine hydroxylase
After citruline

14. central and peripheral demyelination with ataxia and dementia
Vit K antagonist
Actin and myosin
Pseudomonas and s aureus
Metachromatic leukodystrophy - arylsulfatase A - cerebroside sulfate AR

15. What shuttle is involved in fatty acid synthesis and What does it move From where to where
Williams syndrome
Blood - bone marrown - amniotic fluid - placental tissue
Citrate - acetyl coa from mito to cyto
Rb and p53

16. What is chediak higashi
Glycogen phosphorylase
Chronic granulomatous disease - no respiatory burst - no formatino of ROS
Microtuble polymerization defect causing a dec in phagocytosis results in recurrent pyogenic infections - partial albinism - and peripheral neuropathy
Four

17. What amino acid makes up most of the octamer
Elastase - inhibited by alpha1 antitrypsin
Ca/calmodulin in muscle to coordinate with muscle activity
In mitochondria - pyruvate to oxaloacetate - requires biotin - ATP - activated by acetyl coA
Lysine and arginine

18. In which structures do you find microtubules
Williams syndrome
Trisomy 13 - severe mental retardation - rocker bottom feet - micophthlamia - microcephaly - cleft lip/palate - holoprosencephaly - polydactyly - congenital heart disease - death usually within the first year
Inc glucagon - inc cAMP - inc PKA
Flagella - cilia - mitotic spindles

19. Which end of the tRNA is the amino acid bound to...

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20. Delivers dietary TGs to peripheral tissues - delivers cholesterol to liver in the form of remnants - mostly depleted of TGs - secreted by intestinal epith cells
Chylomicrons
F16BP
9+2 arrangement of microtubules
Macrocytic - megaloblastic anemia - no neurologic sx - most common vit def in the US - seen in EtOH and pregs

21. How do microtubules grow and collapse
Grows slowly - collapses quickly
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
Schilling test
Euchromatin

22. What is the most abundant protein in the body
Many generations - both male and female affected - often pleiotropic - can present clinically after puberty
Inhibits RNA polymerase II - found in death cap mushrooms
Collagen
Chronic granulomatous disease - no respiatory burst - no formatino of ROS

23. What is the target of the 3' hydroxyl attack
Phenylalanine hydroxylase
HMG- CoA - brain to 2 molecules of acetyl - Coa - excreted in urine
2 pyruvate - 2ATP - 2NADH - 2H+ - 2H2O
The triphosphate bond

24. What does a defective Cl channel do
Secretion of abnl thick mucus that plugs lungs - pancreas - liver
FMR1 gene - methylation - associated with chromosomal breakage
Oxidative is irreversible
Malonyl coa

25. What clotting factors require vit K
Citrate - acetyl coa from mito to cyto
Mcardle's - skeletal muscle glycogen posphorylase
II - VII - IX - X (1972) protein C and S
2 -4 DNP - aspirin

26. Pts with albinism are at inc risk For what cancer
Fructose via sorbitol dehydrogenase - inc sorbitol leading to osmotic damage as in cataracts - retinopathy - peripheral neuropathy as in diabetes
Changed AA (convservative - new AA is similar in chemical structure)
Skin
Trisomy 18 - severe mental retardation - rocker bottom feet - micrognathia - low set ears - clenched hands - prominent occiput - congenital heart disease - death usually within the first year

27. What happens in the first stage of collagen synthesis - and Where does it happen
Exons
Oxidized hemoglobin precipiated within RBCs
RER - translation of alpha chains - usually Gly-X-Y polypeptide (preprocollagen)
Hartnup disease - dec tryptophan absoprtion - malignant carcinoid syndrome (inc tryptopha metabolism) and INH (dec vit B6)

28. What else can phosphoylate phosphorylase kinase
Cheilosis - inflammation of lips - scaling and fissures at the corner of the mouth - corneal vascularization
Ca/calmodulin in muscle to coordinate with muscle activity
RER
Acetly- CoA - CO2 - NADH

29. ADPKD is associated with What additional conditions
Neg to pos
HDL
Polycystic liver disease - berry aneurysm - mitral valve prolapse
Transmitted through both parents - affected mother may have affected children - affected father will have affected children

30. Patients with chronic granulomatous disease are at increased risk of infection From which kind of organisms
Arg and his inc in histones Which bind negatively charged DNA
Mental retardation - flat facies - prominent epicanthal folds - simian crease - gap between 1st 2 toes - duodenal atresia - congenital heart disease (ASD) - inc risk of All and Alzheimers
Transfers cholesterol from mature HDL to VLDL - IDL and LDL (cholesterol ester transfer protein)
Catalase positive (catalase neg produce H2O2 the cell can use) like S. aureus or aspergillus

31. what disease can cause pellagra
Hypercalciuria - loss of appetite - stupor - seen in sarcoidosis from activation fo vit D by epithelioid macrophage
Neurofibromatosis type 1 (von Recklinghausens disease)
Hartnup disease - dec tryptophan absoprtion - malignant carcinoid syndrome (inc tryptopha metabolism) and INH (dec vit B6)
Anchor muscle fibers - primarily in skeletal and cardiac muscle

32. What does the addition of mannose -6 phosphate do
Targets the proteins for lysosome
Cofactor of carboxyltion enzymes: pyruvate carboxylase - acetate - CoA carboxylase - and propionyl - CoA carboxylase
Immotile cilia due to dynein arm defect - male and femail infertile - bronchiectasis - recurrent sinusitis - associated with situs inversus
Alanine

33. Where do you find elastin and What does it do
Carbomoyl phosphate synthetase I
1- capping on 5' (7- methyguanosine) 2 - polyadenylation on 3' end 3 - splicing out of introns
Lungs - large arteries - elastic ligaments - vocal cords - ligamenta flava - stretch.
ATP and alanine

34. What does SAM do
Rotenone - CN- - antimycin A - CO
Y shaped region along the DNA template where leading nad lagging strands are synthesized
HVA
Transfers methyl units

35. What collagen type is most frequently affected in ehlers danlos and What are common complications
Collagen
Specific glycosylases - AP endonuclease
III - joint dislocation - anuerysms - organ rupture
Presence of both nl and mutated mtDNA resulting in mitochondrial inherited disease

36. What is the activated carrier for electrons
RRNA
Orotic acid to UMP
Ketone - methyl
NADH - NADPH - FADH2

37. How is Lesch Neyhan inherited
Glycogen phosphorylase
Cri du chat
Adipse tissue stores - keton bodies become the main source of energy fo the brain and heart - after these are depleted - vital protein degradation accelerates - leading to organ failure and death
X- linked recessive

38. What does the golgi apparatus do on asparagine
Wobble
Pompe's lysosomal alpha 1 -4 glucosidase (acid maltase) (pompe trashes the pump; heart - liver - muscle)
Acetoacetate and beta hydroxybutyrate
Modifies N- oligosaccharides

39. Name 5 drugs that interfere with nucleotide synthesis
Limit protein diet - benzoate or phenylbutarate (both bind amino acids leading to excretion) can decrease ammonia levels
Production of a recombinant DNA molecule that is self perpetuating - plasmids - selection - restriction enzyme cleavage - tissue mRNA with reverse transcriptase to make cDNA
HDL
Hydroxyurea - inhibits ribonucleotide reductase - 6 MP - blocks de novo purine synthesis - 5 FU inhibits thymidilate synthase - MTX - inhibits DHFR - Trimethroprin inhibits bacterial DHFR

40. What liberates glucose from glucose 6 P
II - VII - IX - X (1972) protein C and S
Glucose 6 phosphatase
Via 1 proprionyl - CoA which can enter the TCA as succinyl - CoA and undergo gluconeogenesis
ATP - citrate

41. In mismatch repair - which strand is recognized and In what hereditary syndrome is this defective
Unmethylated - newly synthesized - HNPCC
Glycolysis - fatty acid synthesis - HMP shunt - protein synthesis (RER) - steroid synthesis (SER)
Cri du chat
Rotenone - CN- - antimycin A - CO

42. What is the activated carrier for phosphoryl
ADEK - dependent on gut (ileum) and pancreas - accumulate in fat and can cause toxicity
ATP
Protective against malaria
FMR1 gene - methylation - associated with chromosomal breakage

43. What substances inhibit phosphofructokinase -1
Breaks down acyl - coa to acetyl coa groups in mito
Plasma membrane - 3Na out - 2K in - ATP on cytoplasmic side
ATP - citrate
Anabolic processes like steroid and fatty acid synthesis - respiratory burst - P-450 - glutathione reductase

44. What drugs can cause folate def
Neural tube
HMG- CoA (HMG- CoA to mevalonate
Phenytoin - MTX - and sulfonamides
Vincritsine/vinblastine

45. RNA poly can't proofread - but What can it do
Initiate chains
Mental retardation - flat facies - prominent epicanthal folds - simian crease - gap between 1st 2 toes - duodenal atresia - congenital heart disease (ASD) - inc risk of All and Alzheimers
Liver hepatocytes and steroid producing cells of the adrenal cortex
THFs

46. What does commaless - nonoverlapping genetic code refer to...
Read from a fixed starting point as a continuous sequence of bases
Core proteins
Ile - phe - thr - trp
In mitochondria - pyruvate to oxaloacetate - requires biotin - ATP - activated by acetyl coA

47. Of the four possible fates for pyruvate - which one can replenish TCA cycle or be used in gluconeogenesis
Glucose 6 phosphate dehydrogenase (G6PD)
Huntingtons
2 rings
Oxalacetate

48. What is the RDE of ketogenesis
NH2-(C=O) - NH2 one NH2 from ammonia the C=O from CO2 - and the other NH2 from aspartate
CarTWOlage
Fructose 1 -6 bisphosphate
HMG- CoA synthase

49. What is locus heterogeneity and give an example
3 NADH - 1 FADH2 - 2 CO2 - 1 GTP = 12 ATP (x2 per glucose)
Dec phenylalanine (contained in aspartame - Nutrasweet) inc tyrosine in diet
Cyclin dependent kinases;constitutive and inactive
Mutations at different loci can produce the same phenotype - marfans - MEN 2B - homocystinuria; all cause marfinoid habitus - also albinism

50. What does vit E def cause
B48 - AIV - CII - E
UGA - UAA and UAG
Inc fragility of RBC - hemolytic anemia - muscle weakness - posterior column and spinocerebellar tract demyelination
Heterochromatin = HighlyCondensed