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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. Severe fasting hypoglycemia - inc glycogen storage in liver - inc blood lactate - hepatomegaly - dz and def enzyme
5' to 3'
Von gierkes - glucose 6 phosphatase
Adds 2 carbon with the help of biotin
Alpha 1 -6 and alpha 1 -4

2. What is the defect in cystinuria
FISH
Hereditary defect of renal tubular amino acid transporter for cystein - ornithine - lysine and arginine in PCT
Zero
F16BP

3. What ribosomes do eukaryotes have
40 - 60 - 80
Each codon specifies only 1 amino acid
RER - translation of alpha chains - usually Gly-X-Y polypeptide (preprocollagen)
Von hippel lindau - 3

4. Which anti breast cancer drugs work on micortubules
Palate - facial and cardiac defects
Inc Cl - in sweat
Paclitaxel
CAG

5. What converts dopamine to NE
CGG
Hyperlipidemia
PCR - denaturation - annealing - elongation
Dopamine beta hydroxylase

6. in a 100 meter sprint Where does energy come from
Krabbes - galactocerebrosidase - galactocerebroside - AR
Fabrys - alpha galactosidase A - ceramide trihexoside - XR
Stored ATP - creatine phosphate - anaerobic glycolysis
Euchromatin

7. Of the four possible fates for pyruvate - which one carries amino groups to liver from muscle
Alanine
Severity of disease worsens or age of onset of disease is earlier in succeeding generations - huntingtons
Mutated dystrophin gene - less severe - adolescence
Cyclin dependent kinases;constitutive and inactive

8. What is disulfiram used for
Orotic acid phosphoribosyltransferase or orotidine 5'- phosphate decarboxylase
Core proteins
Inhibits acetaldehyde dehydrogenase and contributes to hangover symptoms
Dec in alpha ketoacid dehydrogenas leading to blocked degredation of branches amino acids like Ile - Leu - and Val - severe CNS defects mental retardation and death

9. What does a decrease in decrease in NADPH lead to and why
Binds to LDL receptor - mediates VLDL secretion
Riboflavin - cofactor in oxidation and reduction (FAD and FMN are derived from riboflavin)
Poor defense from oxidizing agents - fava beans - sulfonamides - primaquine - antituberclosis drugs leadig to hemolytic anemia.
Lysine and arginine

10. What is the TX for PKU
Reads usual codon but inserts wrong AA
Malapsorption syndromes like sprue or CF or mineral oil intake
Dec phenylalanine (contained in aspartame - Nutrasweet) inc tyrosine in diet
Connective tissue

11. cytoskeletal elements in micorvilli - muscle contracting fibers - cytokenesis - adherens jxns
Carbomoyl phosphate synthetase II
Adds 2 carbon with the help of biotin
CTG
Actin and myosin

12. What is the treatment for orotic aciduria
TTP
Oral uridine administration
Gaucher's - beta glucocerebrosidase - glucocerebrosie - AR
30 - 50 - 70

13. Why can't muscle produce in gluconeogenesis
Disorder of aromatic amino acid metabolism
Lacks glucose 6 phophatase
25OHD3
IDL

14. What is the physiologic role of dystrophin
Alpha 1 -6 and alpha 1 -4
Anchor muscle fibers - primarily in skeletal and cardiac muscle
Promotor - TATA box - and CAAT box - AT rich
HMG- CoA reductase

15. What is imprinting and give an example

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16. How does OTC def present
Proton gradient
ATP
Attachment of ubiquitin to defective proteins tag them for breakdown
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

17. What does a phosphorylase do
Glycogen synthase
Hyperlipidemia
Adds an inorganic phosphate onto substrate without using ATP
P+q = 1

18. decreases In what substances can cause PKU
Phenylalanine hydroxylase - tetrahydrobiopterin cofactor
Hypoglycemia
Diphyllobothrium latum
Chylomicrons

19. What causes B12 def
Facial flushing
SNP
Malabsorption (sprue - enteritis) lack of intrinsic factor from pernicious anemia - gastric bypass surgery or absence of terminal ileum as in Crohns
30 - 50 - 70

20. What does the start codon code for in eukaryotes and prokaryotes
Alpha 1 -4 glucosidase
Eu - methionine - pro - formyl - methionine
Anabolic processes like steroid and fatty acid synthesis - respiratory burst - P-450 - glutathione reductase
Brittle bone disease - most commonly lead AD with abnl type I collagen causing multiple fractures with minimal trauma - blue sclerae due to translucency of connective tissue over the choroid - hearing loss - dental imperfections due to lack of dentin

21. cytoskeletal elements associated with cilia - flagella - mitotic spindle - neurones and centrioles
More than 1 codon may code for the same amino acid
Inc glucagon - inc cAMP - inc PKA
Microtubules
Each codon specifies only 1 amino acid

22. What is the function and name of vit B6
Microtubules
Pompe's lysosomal alpha 1 -4 glucosidase (acid maltase) (pompe trashes the pump; heart - liver - muscle)
9+2 arrangement of microtubules
Pyroxidine - converted to pyroxidine phosphate a cofactor used in transamination - decarboxylation - glycogen phosphorylase - cystathionine synthesis and heme synthesis - required for synthesis of niacin and tryptophan

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

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24. Of the four possible fates for pyruvate - which one is a transition from glycolysis to TCA cycle
High output cardiac failure - dilated cardiomyopathy - edema
Acetyl - CoA
Myeloperoxidase
Actin and myosin

25. what findings are associated with marfans
Orotic acid phosphoribosyltransferase or orotidine 5'- phosphate decarboxylase
Alpha 1 -6 and alpha 1 -4
Tall with long extremeties - pectus excavatum - hyperextensive joints - arachnodactyly
In mitochondria - pyruvate to oxaloacetate - requires biotin - ATP - activated by acetyl coA

26. What substances are uncouling agents
Alpha 1 -4 glucosidase
Fasting
2 -4 DNP - aspirin
Liver and beta cells of pancreas - low affinity - high Km - high capacity - high Vmax - induced by insulin

27. What does commaless - nonoverlapping genetic code refer to...
Mental retardation - macro - orchidism - long face with large jaw - large everted ears - autism - and mitral valve prolapse
Read from a fixed starting point as a continuous sequence of bases
Octamer = 2 sets of H2A - H2B - H3 - H4 - tie =H1
More than 1 codon may code for the same amino acid

28. Who typically has lactase def
African Americans and Asians
Anabolic processes like steroid and fatty acid synthesis - respiratory burst - P-450 - glutathione reductase
Phosphenolpyruvate to pyruvate catalyzed by pyruvate kinase
4 under the floor

29. In addition to fructose and galactose - what sugards should be excluded from the diets of patients with disorders of fructose or galactose metabolism
Animal products - synthesized only by microorganisms - large reserve pool - mainly in liver
Catabolic processes to carry reducing equivalents away as NADH
Sucrose = glucose + fructose - lactose = glucose + galactose
Hartnup disease - dec tryptophan absoprtion - malignant carcinoid syndrome (inc tryptopha metabolism) and INH (dec vit B6)

30. What makes up a nucleoside
Base + ribose
HMG- CoA reductase
Excess ATP - dATP - feeback inhibits ribonucleotide reductase - no DNA synthesis
GTP

31. What does Citrate Is Krebs starting substrate for making oxaloacetate
MRNA - tRNA
Chylomicrons
Q -
Citrate - isocitrate - alpha ketoglutarate - succinyl - CoA - succinate - fumarate - malate - oxaleoacetate

32. Synthesis of vit B3 requires what other vitamin
B6
Tay- Sachs - hexosaminidase A - GM2 ganglioside - AR
BOne
Prevent strands from reannealing

33. What step of uric acid synthesis does xanthine oxidase catalyze
Protein
Hypoxanthine to xanthing and xanthine to uric acid
RRNA
Alpha 1 -4 glucosidase

34. What does vit E def cause
Inc fragility of RBC - hemolytic anemia - muscle weakness - posterior column and spinocerebellar tract demyelination
Citrate - acetyl coa from mito to cyto
NH2-(C=O) - NH2 one NH2 from ammonia the C=O from CO2 - and the other NH2 from aspartate
Euchromatin

35. What does DNA ligase do
Citrate - isocitrate - alpha ketoglutarate - succinyl - CoA - succinate - fumarate - malate - oxaleoacetate
Seals.
SnRNPs
Ubiquitous - low Km - high affinity - low capacity low Vmax - uninduced by insulin

36. What 3 steps in RNA processing occur after transcription

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37. 1 g of protein or cabrohydrate = ?kcal
NADPH
Four
3 NADH - 1 FADH2 - 2 CO2 - 1 GTP = 12 ATP (x2 per glucose)
Epithelial cells

38. What is the amino acid precuros for niacin and serotonin/melatonin
Locus heterogeneity - ocular albinism is x- linked recessive
Males are infertile due to bilateral absence of vas deferens
Tryptophan
Alcohol version of glucose - can trap glucose in cell - aldose reductase

39. How do tetracyclines work
Bind 30s subunit preventing attachment of aminoacyl - tRNA
No mutation occuring at the locus - no selection for any genotypes at the locus - completely random mating - no migration
Aldolase B - AR - fructose 1P accumulates causing a dec in availabel phosphate which inhibts glycogenolysis and gluconeogenesis leading to hypoglycemia - cirrhosis - jaundice and vomiting
Chronic granulomatous disease - no respiatory burst - no formatino of ROS

40. Which anticancer drugs work on microtubules
Anabolic processes like steroid and fatty acid synthesis - respiratory burst - P-450 - glutathione reductase
Riboflavin - cofactor in oxidation and reduction (FAD and FMN are derived from riboflavin)
Mediates chylomicron secretion
Vincritsine/vinblastine

41. What happens do glycogen in skeletal muscle during exercise
Mutations at different loci can produce the same phenotype - marfans - MEN 2B - homocystinuria; all cause marfinoid habitus - also albinism
APC on chromosome 5
B12 and folate
Glycogenolysis to form glucose

42. What happens in folate def
Macrocytic - megaloblastic anemia - no neurologic sx - most common vit def in the US - seen in EtOH and pregs
Fatty acid oxidation - acetyl - Coa production - TCA cycle - OXPHOS
Flank pain - hematuria - HTN - progressive renal failure
NAD+

43. What defects characterize DiGeorge syndrome
Degredation of dietary TG in small intestine
Thymic - parathyroid and cardiac
Octamer = 2 sets of H2A - H2B - H3 - H4 - tie =H1
Creat a nick in the helix to relieave supercoils created during replication

44. What does osteogenesis imperfecta causes and why
Brittle bone disease - most commonly lead AD with abnl type I collagen causing multiple fractures with minimal trauma - blue sclerae due to translucency of connective tissue over the choroid - hearing loss - dental imperfections due to lack of dentin
Arg - lys - his - arg is most basic - has has no charge at body pH
DNA
Inc intestinal absorption fo calcium and phosphate - inc bone resorption

45. What does a defective Cl channel do
Secretion of abnl thick mucus that plugs lungs - pancreas - liver
Cleft palate - cardiac abnl - pregs test
Liver - also in kidney and gut epithelium
Glucose 6P to CO2 - 2NADPH - ribulose 5P - G6PD - rate limiting step

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

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47. What do albinism - ARPKD - CF - glycogen storage diseases - hemochromatosis - mucopolysaccharidoses (except Hunters) PKU - sickle cell - sphingolipodoses (except Fabrys) thalassemias have in common
Hypoxanthine to xanthing and xanthine to uric acid
PMNs
Autosomal recessive diseases
HMP shunt

48. What is the most abundant type of RNA
CTG
RRNA
APC on chromosome 5
Antioxidant - facilitates iron absorption by keeping iron in the Fe2+ reduced state - necessary for hydroxylation of proline and lysine collagen bridges - necessary for dopamine beta hydroxylase which converts DA to NE

49. What enzyme catalyzes the rxn from pyrvuate to Acetyl - CoA and what inhibits it
Oral uridine administration
Inhibits the Na/K pump by binding the K side
Oxidative is irreversible
Pyruvate dehydrogenase - ATP - NADH - acetyl - CoA

50. Which antifungal drugs work on microtubules
Silencers
Griseofulvin
B100 and E
2 -4 DNP - aspirin