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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. phosphorylation of glucose to yield glucose 6 phosphate serves as the 1st step of what rxns and what enzymes catalyze it
Base + ribose + phosphate (3' -5') phosphodiester bond
Glycolysis and the glycogen synthesis in the liver - hexokinase or glucokinase
Binds 50S - blocking translocation
Proton gradient

2. How is ammonium transported from muscle to the liver for urea cycle
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
Silencers
Night blindness - dry skin
Acetly- CoA - CO2 - NADH

3. What apolipoproteins are on chylomicrons
Ketone - methyl
Bone - skin - tendon - dentin - fascia - cornea - late wound repair
B48 - AIV - CII - E
Not all individuals with a mutant genotype show the mutant phenotype

4. In a marathon Where does energy come from
EtOH dehydrogenase and acetaldehyde dehydrogenase
Lebers hereditary optic neuropathy - degeneration of retinal ganglion cells and axons - leads to acute loss of central vision. Mitochondrial myopathies
Glycogen and FFA oxidation; glucose conserved for final sprinting
Adenylyl cycle - inc cAMP - inc PKA - glycogen phosphorylase kinase activated - glycogen phosphoylase active - glycolysis

5. How many rings do pyrimidines have
NAD+
Nature and severity of phenotype vary from 1 individual to another - NF type 1
Promotor - TATA box - and CAAT box - AT rich
1 ring

6. What is the activated carrier for electrons
NADH - NADPH - FADH2
Oxidized hemoglobin precipiated within RBCs
Arginine
ATP and alanine

7. What are the results of pancreatic insuff in CF
NADH - NADPH - FADH2
Unwinds DNA template at replcation fork
Malabsorption and steatorrhea (ADEK)
NAD+

8. What is the treatment for dec affinity of cystathionine synthase for pyroxidal phosphate
HGPRT - defective purine salvage - excess uric acid production
Kwashiorkor - small child with swollen belly
Inc vit B6
UGA - UAA and UAG

9. What causes patau's syndrome and What is it
Gamma carboxylation of glutamic acid residues on various proteins concerned with blood clotting - synthesized in intestinal flora
IMP precursor
Marfans
Trisomy 13 - severe mental retardation - rocker bottom feet - micophthlamia - microcephaly - cleft lip/palate - holoprosencephaly - polydactyly - congenital heart disease - death usually within the first year

10. How do stable (quiescent) cells grow and regenerate and What are examples
SnRNPs
Enter G1 from G0 when stimulated - hepatocytes and lymphocytes
Protein kinase A
FAP

11. How does ouabain work
Inhibits the Na/K pump by binding the K side
Wobble
Mediates chylomicron secretion
Depleted for gluconeogenesis staling the TCA cycle and shunting glucose and FFA to production of ketone bodies

12. What are the teratogenic effects of vit A and What test must be ordered before starting a woman on isotreitinoin for severe acne
Active secretion in lungs and GI - reabsorbs in skin
Plasma membrane - 3Na out - 2K in - ATP on cytoplasmic side
Neg to pos
Cleft palate - cardiac abnl - pregs test

13. What does vit B3 def result in
Met - val - arg his
5' to 3'
Glossitis - severe = pellagra - diarrhea - dermatitis - dementia
Protein kinase A

14. What does high cholesterol or long saturated fatty acid content do to the melting temperature and fluidity
Purines= A - G pyrimidine = C - T (U)
Result from phagocytic removal of heinz bodies my macs - G6PD def
Sucrose = glucose + fructose - lactose = glucose + galactose
Inc melt - dec fluidity

15. decreases In what substances can cause PKU
Malate - aspartate shuttle or the glycerol 3 phosphate shuttle
HGPRT - defective purine salvage - excess uric acid production
Phenylalanine hydroxylase - tetrahydrobiopterin cofactor
LPL def - or altered apoCII - elevated TG and cholesterol - causes pancreatitis - hepatosplenomegaly and eruptive/pruritic xannthomas - no risk of atherosclerosis

16. Describe the replication fork
Neural tube
Type II
Y shaped region along the DNA template where leading nad lagging strands are synthesized
Miscarriage - stillbirth - chromosomal imbalance (down - patau)

17. Which cells are rich in smooth ER
RER - translation of alpha chains - usually Gly-X-Y polypeptide (preprocollagen)
Nature and severity of phenotype vary from 1 individual to another - NF type 1
Liver hepatocytes and steroid producing cells of the adrenal cortex
Wernicke - korsakoff - dry and wet beriberi

18. What happens do glycogen in skeletal muscle during exercise
Krabbes - galactocerebrosidase - galactocerebroside - AR
Creat a nick in the helix to relieave supercoils created during replication
Glycogenolysis to form glucose
Inhibits EtOH dehydrogenase and is an antidote for methanol and ethylene glycol poisoning

19. colon becomes covered witih adenomatous polyps after puberty - progresses to colon cancer unless resected
FAP
Gaucher's - beta glucocerebrosidase - glucocerebrosie - AR
Glucose 6 phosphate dehydrogenase (G6PD)
Cleft palate - cardiac abnl - pregs test

20. Type III collagen
Conversion of NE to epi
Antibiotic use or excessive ingestion of raw eggs
Ribose 5- P to PRPP
Reticulin - skin - blood vessels - uterus - fetal tissue - granulation tissue

21. ADPKD is associated with What additional conditions
Mitochondria
Polycystic liver disease - berry aneurysm - mitral valve prolapse
Degradation of TG remaining in IDL
Via 1 proprionyl - CoA which can enter the TCA as succinyl - CoA and undergo gluconeogenesis

22. What is the defect in II A familial hypercholesterolemia
AD absent of dec LDL receptors causes accelerated atherosclerosis - achilles xanthomas and corneal arcus - increase LDL and elecated cholesterol
SnRNPs and other proteins
Neurofibromatosis type 1 (von Recklinghausens disease)
Common metabolites like pyruvate and acetyl CoA and excess NH4+ converted to urea and exreted

23. The golgi apparatus fxns as a distribution center between what organelles in the cell and What does it process
OTC has hyperammonemia - orotic aciduira does not
Delayed wound healing - hypogonadism - dec in adult hair - dysguesia - anosmia - may predispose to EtOH cirrhosis
Palate - facial and cardiac defects
For proteins and lipids from ER to plasma membrane - lysosomes and secretory vesicles

24. What is the activated carrier for phosphoryl
Excess ATP - dATP - feeback inhibits ribonucleotide reductase - no DNA synthesis
Heterochromatin = HighlyCondensed
Mediates chylomicron secretion
ATP

25. Very Poor Carbohydrate Metabolism stands for 4 of the glycogen storage diseases - What are thy

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26. What is NADPH's role inside RBCs
Hepatic glycogenolysis >> hepatic gluconeogenesis > adipose release of FFA
Scrutinizes - hydrolyzes the bond
Keep glutathione reduced so it can detoxify free radicals and peroxides
Lack of tyrosinase (no melanin) AR ordefective tyrosine transporters (less tyrosine) - can results in lack of migration of NC cells

27. Which antifungal drugs work on microtubules
Histidine
Inc melt - dec fluidity
Loss of brush border enzyme causing bloating - cramps - osmotic diarrhea
Griseofulvin

28. What is chediak higashi
FAP
Microtuble polymerization defect causing a dec in phagocytosis results in recurrent pyogenic infections - partial albinism - and peripheral neuropathy
NH2-(C=O) - NH2 one NH2 from ammonia the C=O from CO2 - and the other NH2 from aspartate
Removal of N or C termal propeptides from zymogens to generate mature proteins

29. What enzyme converts phenylalanine to tyrosin
OTC has hyperammonemia - orotic aciduira does not
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
Phenylalanine hydroxylase
IMP precursor

30. FAP is due to deletion On what gene On what chromosome
Fed
Neuralgia
Prevent strands from reannealing
APC on chromosome 5

31. What causes Marfan syndrome
Type II
Defect in fibrillin
17 - 17 letters in von Recklinghausen
Carbomoyl phosphate synthetase II

32. How do labile celss grow and regenerate and What are examples
Inc NADH/NAD ratio in liver - causing diversion of pyruvate to lactate and OAA to malate - inhibiting gluconeogenesis and stimulating fatty acid synthesis. Leads to hypoglycemia and hepatic fatty liver change seen in chronic EtOH users
Oral uridine administration
Never go to G0 - divide rapidly with a short G1 - bone marrow - gut epi - skin - hair follicles
Alcohol version of glucose - can trap glucose in cell - aldose reductase

33. What is the RDE of glycogen synthesis
Von gierkes - glucose 6 phosphatase
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
CG- 3 > AT-2 - More CG content - melting point goes up
Glycogen synthase

34. What is the Name and function of vit B2
Riboflavin - cofactor in oxidation and reduction (FAD and FMN are derived from riboflavin)
Proton gradient
Elastase - inhibited by alpha1 antitrypsin
MRNA - tRNA

35. What is axonemal dynein
ATPase that links peripheral 9 doublets and causes bending of cilium by differential sliding of doublets
30 - glycerol -3- phosphate shuttle
Liver hepatocytes and steroid producing cells of the adrenal cortex
Degradation of TG stored in adipocytes

36. What is the purpose of the HMP shunt
Alpha1 antitrypsin
Provide a source of NADPH from an abundantly available glucose 6P - create ribose for nucleotide synthesis and glycolytic intermediates
Lebers hereditary optic neuropathy - degeneration of retinal ganglion cells and axons - leads to acute loss of central vision. Mitochondrial myopathies
Malonyl coa (+ biotin= palmitiate - 1 16C fatty acid)

37. What are the mRNA stop codons
Reticulin - skin - blood vessels - uterus - fetal tissue - granulation tissue
Inhibits EtOH dehydrogenase and is an antidote for methanol and ethylene glycol poisoning
VMA
UGA - UAA and UAG

38. What is pseudohypertrophy in the calf a result of - and What are the cardiac manifestations
Fibrofatty replacement of muscle - cardiac myopathy
Citrate - isocitrate - alpha ketoglutarate - succinyl - CoA - succinate - fumarate - malate - oxaleoacetate
BOne
Glucose 6 phosphate dehydrogenase (G6PD)

39. Of the four possible fates for pyruvate - which one carries amino groups to liver from muscle
Heterochromatin = HighlyCondensed
Avidin
Alanine
Protective against malaria

40. What are the findings with homocystinuria and What amino acid is needs to be supplemented
RRNA
HGPRT - defective purine salvage - excess uric acid production
Cyclin dependent kinases;constitutive and inactive
Homocystein in ruine - mental retardation - osteoporosis - tall stature - kyphosis - lens subluxation and atherosclerosis leading to stroke and MI - cysteine

41. What are the two transgenic strategies in mice
Antioxidant - protects RBCs and membrances from free radical damage
Lacks glucose 6 phophatase
Constitutive - random insertion of gene into mouse genome and conditional - targeted insertion or deletion of gene through homologous recombination with mouse gene
Supply sufficient glucose to brain and RBCs and to preserve protein

42. cytoskeletal elements associated with vimenentin - desmin - cytokeratin - glial fibrillary acid protiens (GFAP) - neurofilaments
THFs
Intermediate filaments
Keep glutathione reduced so it can detoxify free radicals and peroxides
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

43. What can excess vit B3 cause
Read from a fixed starting point as a continuous sequence of bases
Flank pain - hematuria - HTN - progressive renal failure
Facial flushing
Glycolysis and the glycogen synthesis in the liver - hexokinase or glucokinase

44. facial lesions - hypopigmented 'ash leaf spots -' cortical and retinal hamartomas - seizures - mental retardation - renal cysts - renal angiomyolipomas - cardiac rhabdomyomas - inc risk of astrocytomas
VMA
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
Tuberous sclerosis
Hypoglycemia

45. What are the priorities for the body in fasting and starvation
Inhibits lipoic acid - vomiting - rice water stools - garlic breath
Supply sufficient glucose to brain and RBCs and to preserve protein
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
Dermatitis - glossitis - and diarrhea

46. delivers hepatic TGs to peripheral tissue - secreted by liver
HGPRT - defective purine salvage - excess uric acid production
Loss of brush border enzyme causing bloating - cramps - osmotic diarrhea
Degredation of dietary TG in small intestine
VLDL

47. Of the four possible fates for pyruvate - which one can replenish TCA cycle or be used in gluconeogenesis
Cystathionine synthase def - dec affinity of cystathionine synthase for pyridoxal phosphate - homocystein methyltransferase def
Transmitted through both parents - affected mother may have affected children - affected father will have affected children
Oxalacetate
Carbomoyl phosphate synthetase I

48. In prokaryotes - What does makes the different types of RNA
Acetyl - CoA
1 kind with multiple subunits
RNA
Degradation of TG remaining in IDL

49. What is the smallest mutation a mircoarray can detect
Von Gierke's - Pompe - Cori - McArdle
Neurofibromatosis type 1 (von Recklinghausens disease)
Mental retardation - growth retardation - seizures - fair skin - eczema - musty body odor
SNP

50. What is the target of the 3' hydroxyl attack
Biotin
Binds to LDL receptor - mediates VLDL secretion
3' end (with CCA)
The triphosphate bond