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Biochemistry

Instructions:

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. What does the golgi do to sugars in proteoglycans and of selected tyrosine on proteins
Uses ATP to add high energy phophate group onto substrate
Sulfation
Carbomoyl phosphate synthetase I
FMR1 gene - methylation - associated with chromosomal breakage

2. Which bond is stronger AT or CG and why? What is the laboratory consequence of this?
CG- 3 > AT-2 - More CG content - melting point goes up
Cyclin dependent kinases;constitutive and inactive
Von Gierke's - Pompe - Cori - McArdle
Bruton's agammaglobulinemia - wiskott - aldrich - fabrys disease - G6PD def - ocular albinism - Lesch - Nyhan syndrome - Duchenne and Becker Muscular Dystrophy - hunter's syndrome - hemophilia A and B

3. What induces pyruvate kinase
F16BP
FMR1 gene - methylation - associated with chromosomal breakage
Supply sufficient glucose to brain and RBCs and to preserve protein
Pantothenate - essential component of CoA - a cofactor for acyl transfers and fatty acid synthase

4. What is the main source of folate
Phosphorylation - glycosylation - hydroxylation
Phenylethamolamine N methyl transferase
Promotor - TATA box - and CAAT box - AT rich
Foliage - small reserve in liver - eat green leaves

5. peripheral neuropathy - developmental delay - optic atrophy - glopoid cells
Krabbes - galactocerebrosidase - galactocerebroside - AR
Protein kinase A
Terminal regions - tropocollagen
Core proteins

6. What substance inside the cells replenishes NADPH
Macrocytic - megaloblastic anemia - no neurologic sx - most common vit def in the US - seen in EtOH and pregs
Myeloperoxidase
TTP
G6PD

7. What is the RDE of cholesterol synthesis
Blood - bone marrown - amniotic fluid - placental tissue
Activates LCAT
HMG- CoA reductase
Disorder of aromatic amino acid metabolism

8. Wgat substances directly inhibit mitochondrial ATPase - causing an inc in proton gradient - no ATP because pump is stopped
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
Removal of N or C termal propeptides from zymogens to generate mature proteins
Oligomycin
Glycogen phosphorylase

9. What is the RDE of fatty acid synthesis
Euchromatin
Never go to G0 - divide rapidly with a short G1 - bone marrow - gut epi - skin - hair follicles
Acetyl - CoA carboxylase (ACC)
Free ribosomes

10. What does a phosphorylase do
Arg and his inc in histones Which bind negatively charged DNA
Stop codon is recognized by release factor - and completed protein is released from ribosome
Adds an inorganic phosphate onto substrate without using ATP
CAG

11. What activates the pyruvate dehydrogenase complex
ATPase that links peripheral 9 doublets and causes bending of cilium by differential sliding of doublets
Disease
Tay- Sachs - hexosaminidase A - GM2 ganglioside - AR
Exercise: inc NAD/NADH - inc ADP - inc Ca

12. How do permanent cells grow and regenerate and What are examples of permanent cells
Remain in G0 - regenerate from stem cells - neurons - skeletal/cardiac muscle - RBCs
Infection - free radicals generated by inflammatory response
Inherited lysosomal storage disorder; failure of addition of mannose 6 phosphate to lysosomal proteins - enzymes secreted outside of cell instead of lysosomes
Binds to LDL receptor - mediates VLDL secretion

13. What does hormone sensitive lipase do
Secretion of abnl thick mucus that plugs lungs - pancreas - liver
Inhibits acetaldehyde dehydrogenase and contributes to hangover symptoms
Degradation of TG stored in adipocytes
Microarrays

14. What is the RDE of glycogen synthesis
Glycogen synthase
Proton gradient
Inhibits 50S peptidyltransferase
Debranching enzyme

15. Which kind of osteogenesis imperfecta is fata in utero or in neonatal period
Asp and Glu
Type II
Common metabolites like pyruvate and acetyl CoA and excess NH4+ converted to urea and exreted
PFK - rate limiting enzyme

16. What is the RDE of the HMP shunt
Dec methionine - inc cystiene - inc B12/folate
Cystine kidney stones - cystine staghorn calculi - cystine is made of two cysteines connected by disulfide bond
ATPase that links peripheral 9 doublets and causes bending of cilium by differential sliding of doublets
Glucose 6 phosphate dehydrogenase (G6PD)

17. Which anti breast cancer drugs work on micortubules
Paclitaxel
Hypoglycemia
No - its non homologous
ADEK - dependent on gut (ileum) and pancreas - accumulate in fat and can cause toxicity

18. In which structures do you find microtubules
Flagella - cilia - mitotic spindles
Cleft palate - cardiac abnl - pregs test
Glucose 6 phosphatase
Mitochondria

19. What substance inside the cell reduces glutatione
Glycolysis and aerobic respiration
Glycogenolysis to form glucose
NADPH
Microtuble polymerization defect causing a dec in phagocytosis results in recurrent pyogenic infections - partial albinism - and peripheral neuropathy

20. progressive neurodegeneration - hepatosplenomegaly - cherry red spot on macula - foam cells
Particular sequence of DNA where replicatino begins - may be single of multiple
Neimann - pick - sphingomyelinase - sphingomyelin - AR
No
Assistance of upper extremities to stand up

21. Describe the location and fxn of the Na/K ATPase
Retardation - self mutilation - aggression - hyperuricemia - gout - choreoathetosis
LCAT (lecithin cholesterol acyltransferase)
Excess ATP - dATP - feeback inhibits ribonucleotide reductase - no DNA synthesis
Plasma membrane - 3Na out - 2K in - ATP on cytoplasmic side

22. How does insulin inhibit glycogenolysis
Transmitted only through mother - all offspring of affected females may show signs of disease
TRNA
Receptor tyrosine kinases - protein phosphatase - takes phosphate off glycogen phosphorylase kinase inactivating it
Supply sufficient glucose to brain and RBCs and to preserve protein

23. What is the activated carrier for electrons
Makes RNA primer on which DNA poly III can initiate replication
Ornithin transcarbamoylase def - x linked recesssive - other urea cycle enzymes defs are autosommal recessive
NADH - NADPH - FADH2
RNA poly II

24. What feedback inhibits hexokinase
Peroxide
Glucose -6 phosphate
CTG
5' to 3'

25. Where is vit B12 found
Cofactor of carboxyltion enzymes: pyruvate carboxylase - acetate - CoA carboxylase - and propionyl - CoA carboxylase
2 pyruvate - 2ATP - 2NADH - 2H+ - 2H2O
MRNA - tRNA
Animal products - synthesized only by microorganisms - large reserve pool - mainly in liver

26. Mild Hurlurs + aggressive behavior no corneal clouding
ATP and methionine
Hunters - iduronate sulfatase - heparan sulfate/dermatan sulfate - XR
Cofactor of carboxyltion enzymes: pyruvate carboxylase - acetate - CoA carboxylase - and propionyl - CoA carboxylase
CG- 3 > AT-2 - More CG content - melting point goes up

27. Which enzyme involved in RNA synthesis does not require a template
Malonyl coa
Poly A polymerase - signal is AAUAA
3' end (with CCA)
Conversion of NE to epi

28. What happens do glycogen in skeletal muscle during exercise
Glycogenolysis to form glucose
Dopamine beta hydroxylase
Mental retardation - hyperphagia - obesity - hypogonadism - hypotonia - deletion of normally active paternal allele on chromosome 15
II - VII - IX - X (1972) protein C and S

29. What happens with wet beriberi
UGA - UAA and UAG
High output cardiac failure - dilated cardiomyopathy - edema
Oxidative is irreversible
Via 1 proprionyl - CoA which can enter the TCA as succinyl - CoA and undergo gluconeogenesis

30. Where is acetaldehyde located
Activates LCAT
Connective tissue
Mitochondria
Von Gierke's - Pompe - Cori - McArdle

31. I g fat = ? Kcal
THFs
Nine
Protein
Avidin

32. What substance inside the cell serves to oxidize glutatione
Peroxide
Von Gierke's - Pompe - Cori - McArdle
Neonatal hemorrhage with inc PT and PTT - but nl bleeding time - can also occur after prolonged use of broad spectrum Abx
Isocitrate dehydrogenase

33. What creates the musty body odor in PKU
Disorder of aromatic amino acid metabolism
Acetyl - CoA to malonyl - CoA (2C to 3C)
Fabrys - alpha galactosidase A - ceramide trihexoside - XR
Ribos first then deoxyribos with ribonucleotide reductase

34. What are the only purely ketogenic amino acids
Inability to transport LCFAs into mitochondria results in toxic accumulation causing weakness - hypotonia - hyperketotic hyperglycemia
Heme synthesis - urea cycle and gluconeogenesis - HUGs take two
Lysine and leucine
Many generations - both male and female affected - often pleiotropic - can present clinically after puberty

35. What does beta oxidation do and Where does it occur
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
Breaks down acyl - coa to acetyl coa groups in mito
2 rings
Coarse facial features - clouded corneas - restricted joint movements - high plasma levels of lysosomal enzymes - often fatal in childhood

36. What is the limiting reagent in EtOH metabolism
NAD+
HDL
Pyruvate dehydrogenase - ATP - NADH - acetyl - CoA
Glycine - aspartate - glutamine

37. spheroid RBCs due to spectrin or ankyrin defect - hemolytic anemia - inc MHCH - splenectomy is curative
Hereditary spherocytosis
Lactate
Phenylalanine hydroxylase
Macrocytic - megaloblastic anemia - hypersegmented PMNs - neurologic symptoms - (parasthesias - subacute combined degeneration) due to abnl myelin - prolonged becomes irreversible

38. What is the energy source for translocation
Degredation of dietary TG in small intestine
Reads usual codon but inserts wrong AA
GTP
Same AA - often base change in 3rd position of codon (tRNA wobble)

39. How do cardia glycosides work
APKD1 on chromosome 16
Sons of heterozygous mothers have 1/2 half chance of being affected - no male to male transmission - and often more severe in males - heterozygous females may be affected
In cytosol - oxaloacetate to phosphenolpyruvate - requires GTP
Directly inhibit the Na/K pump which leads to indirect inhibition of Na/Ca exchange - inc calcium inside the cell - inc cardiac contractility

40. What is the TX for hyper ammonemia
Inc fragility of RBC - hemolytic anemia - muscle weakness - posterior column and spinocerebellar tract demyelination
Limit protein diet - benzoate or phenylbutarate (both bind amino acids leading to excretion) can decrease ammonia levels
AMP - fructose 2 -6 BP
Protein

41. several distinct syndromes characterized by familial tumors of endocrine glands including pancreas - parathyroid - pituitary - thryoid and adrenal medulla - disorders and gene association
HMG- CoA reductase
No
MEN - 2A and 2B with ret gene
Lacks glucose 6 phophatase

42. What is the RDE of fatty acid oxidation
Abnormal protein folding - degradation before reaching cell surface
Phenylethamolamine N methyl transferase
More than 1 codon may code for the same amino acid
Carnitine acyltransferase I

43. What are the reactants for glycolysis
Receptor tyrosine kinases - protein phosphatase - takes phosphate off glycogen phosphorylase kinase inactivating it
Glucose - 2Pi - 2ADP - 2NAD+
By inhibiting formation of the initiation complex and cause misreading of mRNA
Isocitrate dehydrogenase

44. What is the energy source after day 3 of starvation
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
Base + ribose
Comlex II
Fasting

45. What 3 amino acids are necessary for purine synthesis
Glycine - aspartate - glutamine
Glycogenolysis to form glucose
Inc melt - dec fluidity
2 rings

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

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47. What kind of branches do glycogen branches have
Carnitine shuttle - acyl - coa from cyto to mito
Alpha 1 -6 and alpha 1 -4
Mebendazole/thiabendazole
25OHD3

48. What substances induce phosphofructokinase
Protein
No mutation occuring at the locus - no selection for any genotypes at the locus - completely random mating - no migration
Nine
AMP - fructose 2 -6 BP

49. What does a decrease in decrease in NADPH lead to and why
G6PD
Poor defense from oxidizing agents - fava beans - sulfonamides - primaquine - antituberclosis drugs leadig to hemolytic anemia.
Modifies N- oligosaccharides
Trisomy 13 - severe mental retardation - rocker bottom feet - micophthlamia - microcephaly - cleft lip/palate - holoprosencephaly - polydactyly - congenital heart disease - death usually within the first year

50. What are the mRNA stop codons
UGA - UAA and UAG
Plasma membrane - 3Na out - 2K in - ATP on cytoplasmic side
Hurlers syndrome - alpha L iduronidase - heparan sulfate/dermatan sulfate - AR
Activates LCAT