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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. What causes Marfan syndrome
Carbomoyl phosphate synthetase II
Locus heterogeneity - ocular albinism is x- linked recessive
Defect in fibrillin
Adds 2 carbon with the help of biotin

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

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3. What does a dehydrogenase do
AD absent of dec LDL receptors causes accelerated atherosclerosis - achilles xanthomas and corneal arcus - increase LDL and elecated cholesterol
Homocystein in ruine - mental retardation - osteoporosis - tall stature - kyphosis - lens subluxation and atherosclerosis leading to stroke and MI - cysteine
FMR1 gene - methylation - associated with chromosomal breakage
Oxidizes substrate

4. What happens in vit D def
Site of steroid synthesis and detoxification of drugs and poisons
Rickets in kids - bending bones - osteomalacia in adults - hypocalcemia tetany - breast milk has dec vit D (supplement in dark skinned patients)
Athralgias - fatigue - headaches - skin changes - sore throat - alopecia
Post to neg

5. What liberates glucose from glucose 6 P
Glucose 6 phosphatase
Transmitted through both parents - affected mother may have affected children - affected father will have affected children
Exercise: inc NAD/NADH - inc ADP - inc Ca
Miscarriage - stillbirth - chromosomal imbalance (down - patau)

6. What happens in hyperammonemia
Tyrosine
Glycogen and FFA oxidation; glucose conserved for final sprinting
Depletes alpha - ketoglutarate leading to inhibition of TCA cycle - tremor slurring of speech - somnolence - vomiting - cerebral edema - blurring of vision
Antioxidant - protects RBCs and membrances from free radical damage

7. What clotting factors require vit K
Glycogenolysis to form glucose
Fatty acid oxidation - acetyl - Coa production - TCA cycle - OXPHOS
II - VII - IX - X (1972) protein C and S
Inhibit DNA gyrase specific for prokaryotic topoisomerase

8. what happens in acyl coa dehyrdogenase def
Phenylalanine hydroxylase - tetrahydrobiopterin cofactor
Ribose 5- P to PRPP
Phenylalanine hydroxylase
Inc dicarboxylic acids - dec in glucose and ketones

9. What is the RDE of gluconeogenesis
Inc dicarboxylic acids - dec in glucose and ketones
Rb and p53
Fructose 1 -6 bisphosphate
ATP

10. What does a phosphorylase do
Adds an inorganic phosphate onto substrate without using ATP
Same as sprint + OXPHOS
ATP
Tall with long extremeties - pectus excavatum - hyperextensive joints - arachnodactyly

11. What converts tyrosine to DOPA
Cheilosis - inflammation of lips - scaling and fissures at the corner of the mouth - corneal vascularization
Phenylketones in urine
Aldolase B - AR - fructose 1P accumulates causing a dec in availabel phosphate which inhibts glycogenolysis and gluconeogenesis leading to hypoglycemia - cirrhosis - jaundice and vomiting
Tryosine hydroxylase

12. What is the exception to genetic redundancy
Excess NADH shunts oxaloacetate to malate (backwards) stalling the TCA and shunting glucose and FFA to ketone body production
5' of the incoming nucleotide
Methionine encode by only 1 codon (AUG)
30 - 50 - 70

13. What does the passage of electrons result in that when coupled to OXPHOS drives the production of ATP
Nine
Macrocytic - megaloblastic anemia - no neurologic sx - most common vit def in the US - seen in EtOH and pregs
Proton gradient
Inability to transport LCFAs into mitochondria results in toxic accumulation causing weakness - hypotonia - hyperketotic hyperglycemia

14. What else can phosphoylate phosphorylase kinase
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
Hurlers syndrome - alpha L iduronidase - heparan sulfate/dermatan sulfate - AR
Makes RNA primer on which DNA poly III can initiate replication
Ca/calmodulin in muscle to coordinate with muscle activity

15. Why can't muscle produce in gluconeogenesis
Poor defense from oxidizing agents - fava beans - sulfonamides - primaquine - antituberclosis drugs leadig to hemolytic anemia.
De novo pyrimidine synthesis and urea - ornithine transcarbamoyl def in the urea cycle leads to a build of CP - then converted to orotic acid in pyrimidine synthesis pathway
Lacks glucose 6 phophatase
After day 1

16. Which type of chromatin is less condensed - transcriptionally active - sterically accesible
Depletes alpha - ketoglutarate leading to inhibition of TCA cycle - tremor slurring of speech - somnolence - vomiting - cerebral edema - blurring of vision
Essential for the activity of over 100+ enzymes - important in the formatio of zinc fingers -
Euchromatin
Miscarriage - stillbirth - chromosomal imbalance (down - patau)

17. How does abetalipoproteinemia present and What is the defect
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
Occurs when cells in the body have different genetic makeup - can be germline mosaic - which may produce a disease that is not carried by parents somatic cells - lyonization - random X inactivation in females
Autosomal recessive diseases
Microtuble polymerization defect causing a dec in phagocytosis results in recurrent pyogenic infections - partial albinism - and peripheral neuropathy

18. What is the energy source for translocation
Metanephrine
3 NADH - 1 FADH2 - 2 CO2 - 1 GTP = 12 ATP (x2 per glucose)
GTP
Inhibits lipoic acid - vomiting - rice water stools - garlic breath

19. What is the amino acid precuros for niacin and serotonin/melatonin
Microarrays
FMR1 gene - methylation - associated with chromosomal breakage
Methionine encode by only 1 codon (AUG)
Tryptophan

20. Give an example of a mitochondrial inherited disease
Lebers hereditary optic neuropathy - degeneration of retinal ganglion cells and axons - leads to acute loss of central vision. Mitochondrial myopathies
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
Carbomoyl phosphate synthetase I
Cytosol

21. The golgi apparatus fxns as a distribution center between what organelles in the cell and What does it process
For proteins and lipids from ER to plasma membrane - lysosomes and secretory vesicles
Dec DNA - dec lymphos leads to SCID
Tyrosine
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

22. colon becomes covered witih adenomatous polyps after puberty - progresses to colon cancer unless resected
FAP
Prokaryotic only - degrades RNA primer and fills in the gap with DNA
Medial dorsal nucleus of thalamus - mamillary bodies
Retardation - self mutilation - aggression - hyperuricemia - gout - choreoathetosis

23. What 3 steps in RNA processing occur after transcription

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24. What is the activated carrier for Co2
OTC has hyperammonemia - orotic aciduira does not
Oral uridine administration
Biotin
SnRNPs

25. What is the source of energy in the fasting state between meals
Hepatic glycogenolysis >> hepatic gluconeogenesis > adipose release of FFA
ATP
Remain in G0 - regenerate from stem cells - neurons - skeletal/cardiac muscle - RBCs
Leu - lys

26. What activates the pyruvate dehydrogenase complex
Hereditary hemorrhagic telangiectasia (Osler - Weber - Rendu syndrome)
Exercise: inc NAD/NADH - inc ADP - inc Ca
Binds to LDL receptor - mediates VLDL secretion
Heterochromatin = HighlyCondensed

27. what disease can cause pellagra
Hartnup disease - dec tryptophan absoprtion - malignant carcinoid syndrome (inc tryptopha metabolism) and INH (dec vit B6)
Abnormal protein folding - degradation before reaching cell surface
II - VII - IX - X (1972) protein C and S
Inhibit DNA gyrase specific for prokaryotic topoisomerase

28. How many ATP are created by aerobic metobolism of glucose in muslce via what shuttle
Prokaryotic only - degrades RNA primer and fills in the gap with DNA
APKD1 on chromosome 16
30 - glycerol -3- phosphate shuttle
Fabrys - alpha galactosidase A - ceramide trihexoside - XR

29. What shape does the RNA generate during splicing and why?
Myeloperoxidase
Lariat shape in order and remove intron precisely and join 2 exons
Hepatic overproduction of VLDL causing pancreatitis - elvelated TGs and VLDL
Lysine and leucine

30. What is the TX for CF and What does it do
N- acteylcysteine - cleave disulfide bonds within mucus glycoproteins
THFs
9+2 arrangement of microtubules
Flank pain - hematuria - HTN - progressive renal failure

31. What does primase do
2pq
Makes RNA primer on which DNA poly III can initiate replication
Phenytoin - MTX - and sulfonamides
Oxidative and nonoxidative - no ATP produced or used

32. What does DNA poly III do?

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33. Giving folate during early pregnancy is important to prevent what birth defects
Phosphorylation - glycosylation - hydroxylation
MRNA - tRNA
Neural tube
2 pyruvate - 2ATP - 2NADH - 2H+ - 2H2O

34. What do DNA topoisomerases do
Impaired glucose breakdown leading to ATP depletion; highly affected aerobic tissues like brain and heart affected first
ADPKD
Creat a nick in the helix to relieave supercoils created during replication
Deamination

35. antbiotic controlled promoter to induciblly manipulate genes at specific developmental points
Cre - lox system
Nature and severity of phenotype vary from 1 individual to another - NF type 1
Malate - aspartate shuttle or the glycerol 3 phosphate shuttle
Grows slowly - collapses quickly

36. What do B- complex vitamin deficiencies often result in
Inc CPK and muscle biopsy
Carnitine acyltransferase I
Dermatitis - glossitis - and diarrhea
75 to 90 - cloverleaf

37. What is made fir rNucleotides or deoxyrNucleotides and what enzyme is necessary to convert one to the next
Cystathionine synthase def - dec affinity of cystathionine synthase for pyridoxal phosphate - homocystein methyltransferase def
Breaks down acyl - coa to acetyl coa groups in mito
Ribos first then deoxyribos with ribonucleotide reductase
Kwashiorkor - small child with swollen belly

38. Wgat substances directly inhibit mitochondrial ATPase - causing an inc in proton gradient - no ATP because pump is stopped
Depletes alpha - ketoglutarate leading to inhibition of TCA cycle - tremor slurring of speech - somnolence - vomiting - cerebral edema - blurring of vision
Oligomycin
III - joint dislocation - anuerysms - organ rupture
Robertsonian translocation and mosaicism

39. Broadly - What can cause fat - soluble vitamin deficiencies
Essential for the activity of over 100+ enzymes - important in the formatio of zinc fingers -
Malapsorption syndromes like sprue or CF or mineral oil intake
Sucrose = glucose + fructose - lactose = glucose + galactose
Fasting

40. What initiates protein synthesis
GTP hydrolysis - initiation factors assemble 40S ribosomal subunit with initiator tRNA and are released with mRNA and ribosomal subunit assemble with the complex
Differences in phenotype depend on whether the mutation is of maternal or paternal origin - prader - willi and angelman's syndrome
Modifies N- oligosaccharides
Hereditary defect of renal tubular amino acid transporter for cystein - ornithine - lysine and arginine in PCT

41. What are the purely ketogenic amino acids
Inc glucagon - inc cAMP - inc PKA
Leu - lys
Supply sufficient glucose to brain and RBCs and to preserve protein
Bind 30s subunit preventing attachment of aminoacyl - tRNA

42. In a marathon Where does energy come from
Glycogen and FFA oxidation; glucose conserved for final sprinting
Inc glucagon - inc cAMP - inc PKA
Eu - methionine - pro - formyl - methionine
II - VII - IX - X (1972) protein C and S

43. peripheral neuropathy of hands/feet - angiokeratomas - CV/renal disease - dz - def enzyme - acc substrate - inherit
Actin and myosin
Fabrys - alpha galactosidase A - ceramide trihexoside - XR
Rb and p53
PMNs

44. What does biotin def cause
Stored ATP - creatine phosphate - anaerobic glycolysis
Abnormal protein folding - degradation before reaching cell surface
Cre - lox system
Dermatitis - alopecia - enteritis

45. A small proportion of Down syndrome is due to What two genetic events
Impaired glucose breakdown leading to ATP depletion; highly affected aerobic tissues like brain and heart affected first
Adenylyl cycle - inc cAMP - inc PKA - glycogen phosphorylase kinase activated - glycogen phosphoylase active - glycolysis
O- oligosaccharaides
Robertsonian translocation and mosaicism

46. What is the mutation in beckers muscular dystrophy - What is the severity and time of onset
Mutated dystrophin gene - less severe - adolescence
Cyclin dependent kinases;constitutive and inactive
Palate - facial and cardiac defects
Chylomicrons

47. Why does alpha amanitin cause liver failure and Where is it found
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
Inhibits RNA polymerase II - found in death cap mushrooms
Keep glutathione reduced so it can detoxify free radicals and peroxides
Avidin

48. What does the golgi apparatus do on asparagine
Essential fructosuria - fructokinase AR
GAA
Modifies N- oligosaccharides
Makes RNA primer on which DNA poly III can initiate replication

49. In which state is FBPase -2 active
Phosphorylation - glycosylation - hydroxylation
Rb and p53
UDP glucose pyrophosphorylase
Fasting

50. What does a kinase do
Neurofibromatosis type 1 (von Recklinghausens disease)
Glutamate
Backup of substrate (pyruvate and alanine) resulting in lactic acidosis - congenital or acquired from thiamine def in EtOH - neuro defects
Uses ATP to add high energy phophate group onto substrate