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Test your basic knowledge |
Biochemistry
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Subjects
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health-sciences
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science
Instructions:
Answer 50 questions in 15 minutes.
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study here
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Match each statement with the correct term.
Don't refresh. All questions and answers are randomly picked and ordered every time you load a test.
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 is the treatment for dec affinity of cystathionine synthase for pyroxidal phosphate
Inc vit B6
Kidney - ears - eyes
Inhibits 50S peptidyltransferase
Citrate - acetyl coa from mito to cyto
2. What is the pathway in the fed state leading to dec FBPase -2 and inc PFK-2
Rotenone - CN- - antimycin A - CO
Same as sprint + OXPHOS
Four
Inc insulin - dec cAMP - dec PKA
3. What is the physiologic role of dystrophin
Hypoxanthine to xanthing and xanthine to uric acid
Anchor muscle fibers - primarily in skeletal and cardiac muscle
Homocystein in ruine - mental retardation - osteoporosis - tall stature - kyphosis - lens subluxation and atherosclerosis leading to stroke and MI - cysteine
Dopa decarboxylase
4. What are the 2 distinct phases of the HMP shunt and How many ATP are used and produced
Oxidative and nonoxidative - no ATP produced or used
Alanine
Fatty acid oxidation - acetyl - Coa production - TCA cycle - OXPHOS
Anabolic processes as a supply of reducing equivalents
5. Where are the secretory proteins made and N- linked oligosaccharide addtions put on proteins
HMG- CoA synthase
RER
Only processed RNA
RRNA
6. What does hormone sensitive lipase do
Catabolic processes to carry reducing equivalents away as NADH
Cystathionine synthase def - dec affinity of cystathionine synthase for pyridoxal phosphate - homocystein methyltransferase def
Degradation of TG stored in adipocytes
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
7. What defects characterize velocardiofacial syndrome
Absence of galactose 1 phosphate uridyltransferase - accumulation of toxic substances leads to failure to thrive - jaundice - hepatomegaly - infantile cataracts - mental retardation
Palate - facial and cardiac defects
Williams syndrome
Stored and undergoes glycogenolysis to maintain blood sugar at appropriate levels
8. What are the findings in Lesch - Nyhan
B(1 - 2 - 3 - 5 - 6 - 12) C - biotin and folate (B12 and folate are stored)
TRNA
Robertsonian translocation and mosaicism
Retardation - self mutilation - aggression - hyperuricemia - gout - choreoathetosis
9. What does DNA poly III do?
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10. What does the mutation in the gene cause in protein synthesis
Abnormal protein folding - degradation before reaching cell surface
Phosphofructokinase 1
Transmitted through both parents - affected mother may have affected children - affected father will have affected children
Nine
11. When does aspartate enter the urea cycle
Thymic - parathyroid and cardiac
After citruline
RNA poly II
SNP
12. What happens in folate def
Cyclin dependent kinases;constitutive and inactive
Neuralgia
Macrocytic - megaloblastic anemia - no neurologic sx - most common vit def in the US - seen in EtOH and pregs
RER
13. What converts tyrosine to DOPA
SnRNPs and other proteins
CTG
Tryosine hydroxylase
NADPH
14. What do the single stranded binding proteins do
Phenylalanine hydroxylase - tetrahydrobiopterin cofactor
Post to neg
Prevent strands from reannealing
Inc fragility of RBC - hemolytic anemia - muscle weakness - posterior column and spinocerebellar tract demyelination
15. What does beta oxidation do and Where does it occur
Inability to transport LCFAs into mitochondria results in toxic accumulation causing weakness - hypotonia - hyperketotic hyperglycemia
Breaks down acyl - coa to acetyl coa groups in mito
1 ring
Glycogen phosphorylase
16. What is the active form of vit D
1 -25 OH2 D3 = calcitriol
ATP
Lysine and leucine
Conversion of NE to epi
17. In what direction are DNA and RNA synthesized
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18. In what cells do the respiratory burst occur
1 gene had >1 effect on an individuals phenotype - PKU causes many seemingly unrelated symptoms ranging from mental retardation to hair/skin changes
Malonyl coa (+ biotin= palmitiate - 1 16C fatty acid)
Y shaped region along the DNA template where leading nad lagging strands are synthesized
PMNs
19. What does a southern blot use as its sample
Mental retardation - hyperphagia - obesity - hypogonadism - hypotonia - deletion of normally active paternal allele on chromosome 15
DNA
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
20. What are bite cells and when do you see them
Result from phagocytic removal of heinz bodies my macs - G6PD def
Cytosol
Dermatitis - alopecia - enteritis
Vit K antagonist
21. caf
Glycolysis - fatty acid synthesis - HMP shunt - protein synthesis (RER) - steroid synthesis (SER)
Neurofibromatosis type 1 (von Recklinghausens disease)
Specific glycosylases - AP endonuclease
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
22. What does NADPH oxidase deficiency result in and why
Superoxide dismutase
Dermatitis - glossitis - and diarrhea
Chronic granulomatous disease - no respiatory burst - no formatino of ROS
3 NADH - 1 FADH2 - 2 CO2 - 1 GTP = 12 ATP (x2 per glucose)
23. Type II collagen
AR
Tall with long extremeties - pectus excavatum - hyperextensive joints - arachnodactyly
Alcohol version of glucose - can trap glucose in cell - aldose reductase
Cartilage - hyaline - vitreous body - nucleus pulposus
24. What components come together to make S- adenosyl methionine
Poor defense from oxidizing agents - fava beans - sulfonamides - primaquine - antituberclosis drugs leadig to hemolytic anemia.
Oxidative and nonoxidative - no ATP produced or used
ATP and methionine
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
25. FAP is due to deletion On what gene On what chromosome
Males are infertile due to bilateral absence of vas deferens
THFs
APC on chromosome 5
Wrinkles and acne
26. What is the Name and fxn of vit B12
Prokaryotic only - degrades RNA primer and fills in the gap with DNA
Stop codon is recognized by release factor - and completed protein is released from ribosome
1/4 of offspring from 2 carrier parents are affected - often enzyme def - usually only seen in 1 generation - often more sever than AD - presents in childhood
Cobalamin - cofactor of homocysteine and methyltransferase - methylmalonyl CoA mutase
27. Give an example of a mitochondrial inherited disease
Cobalamin - cofactor of homocysteine and methyltransferase - methylmalonyl CoA mutase
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
Lebers hereditary optic neuropathy - degeneration of retinal ganglion cells and axons - leads to acute loss of central vision. Mitochondrial myopathies
Changed AA (convservative - new AA is similar in chemical structure)
28. What is the RDE of the HMP shunt
IDL
Glucose 6 phosphate dehydrogenase (G6PD)
AR
Liver - also in kidney and gut epithelium
29. What is the treatment for cystathionine synthase def
Dec methionine - inc cystiene - inc B12/folate
MEN - 2A and 2B with ret gene
Depletes alpha - ketoglutarate leading to inhibition of TCA cycle - tremor slurring of speech - somnolence - vomiting - cerebral edema - blurring of vision
RRNA
30. What other complex is similar to the pyruvate dehydrogenase complex in that it has the same co - factors and generates succinyl - CoA
Niacin - constituent of NAD and NADP - derived from tryptophan
RNA poly II
Alpha - ketoglutarate dehydrogenase complex
Glycogen synthase
31. What does the primary transcript combine with to form the spliceosome
Blood - bone marrown - amniotic fluid - placental tissue
SnRNPs and other proteins
Inhibits RNA polymerase II - found in death cap mushrooms
Inhibits 50S peptidyltransferase
32. How does cytosine become uracil
DTMP
Karyotyping
Deamination
Offspring receives 2 copies of a chromosome from 1 parent and no copies from the other parent
33. What is the longest time of RNA and shortest
Fabrys - alpha galactosidase A - ceramide trihexoside - XR
Binds 50S - blocking translocation
Rotenone - CN- - antimycin A - CO
MRNA - tRNA
34. What is dominant negative mutation and give an example
Exerts a dominant effect - a heterozygote produces a nonfxnal altered protein that also prevents the normal gene from functioning - mutation of Tx factor its allosteric site - nonfxning mutant can still bind DNA - preventing wild type Tx from binding
Terminal regions - tropocollagen
Autosomal recessive diseases
Neither of 2 alleles is dominant - blood groups
35. Where is fructose 1 -6 bisphosphatase found and What does it do
GAA
Cytosol - F 1 -6 BP to fructose 6 Phosphate
Absence of galactose 1 phosphate uridyltransferase - accumulation of toxic substances leads to failure to thrive - jaundice - hepatomegaly - infantile cataracts - mental retardation
Ile - phe - thr - trp
36. How is ATP used by the cell
Hurlers syndrome - alpha L iduronidase - heparan sulfate/dermatan sulfate - AR
Liver - also in kidney and gut epithelium
ATP hyrdolysis couple to energetically unfavorable rxns
Type II
37. Type II cartilage
CarTWOlage
Result from phagocytic removal of heinz bodies my macs - G6PD def
By inhibiting formation of the initiation complex and cause misreading of mRNA
Liver - ovaries - seminal vesicles
38. Type IV BM
Delayed wound healing - hypogonadism - dec in adult hair - dysguesia - anosmia - may predispose to EtOH cirrhosis
HGPRT - defective purine salvage - excess uric acid production
Dopa decarboxylase
4 under the floor
39. What inhibits pyruvate kinase
Euchromatin
25OHD3
ATP and alanine
HMG- CoA reductase
40. What happens in elongation of protein synthesis
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41. What is the energy source in the fed state right after a meal
Epithelial cells
No
PMNs
Glycolysis and aerobic respiration
42. What does the deletion of the dystrophin gene lead to...
AR disorder characterized by defective neutral amino acid transporte on renal and intestinal epith cells
Pyruvate dehydrogenase - ATP - NADH - acetyl - CoA
Accelearted muscle breakdown
LCAT (lecithin cholesterol acyltransferase)
43. Infection with what organism can cause B12 def
Diphyllobothrium latum
Cori's - debranching enzyme alpha 1 -6 glucosidase - gluconeogenesis intact
Griseofulvin
Phosphenolpyruvate to pyruvate catalyzed by pyruvate kinase
44. What tissues have both enzymes of sorbitol metabolism
Glucose - 2Pi - 2ADP - 2NAD+
AR
Liver - ovaries - seminal vesicles
No
45. What is the defect in I- hyperchylomicronemia
Supply sufficient glucose to brain and RBCs and to preserve protein
LPL def - or altered apoCII - elevated TG and cholesterol - causes pancreatitis - hepatosplenomegaly and eruptive/pruritic xannthomas - no risk of atherosclerosis
Males are infertile due to bilateral absence of vas deferens
Homocystein in ruine - mental retardation - osteoporosis - tall stature - kyphosis - lens subluxation and atherosclerosis leading to stroke and MI - cysteine
46. what findings are associated with marfans
HMG- CoA synthase
Tall with long extremeties - pectus excavatum - hyperextensive joints - arachnodactyly
Rotenone - CN- - antimycin A - CO
AR disorder characterized by defective neutral amino acid transporte on renal and intestinal epith cells
47. What metabolic rxns occur in both the cytoplasm and mitochondria
Debranching enzyme
Reticulin - skin - blood vessels - uterus - fetal tissue - granulation tissue
Heme synthesis - urea cycle and gluconeogenesis - HUGs take two
Transmitted only through mother - all offspring of affected females may show signs of disease
48. In a marathon Where does energy come from
Degradation of TG stored in adipocytes
Lacks glucose 6 phophatase
O- oligosaccharaides
Glycogen and FFA oxidation; glucose conserved for final sprinting
49. What is Gowers maneuver
Assistance of upper extremities to stand up
Oxidative and nonoxidative - no ATP produced or used
Niacin - constituent of NAD and NADP - derived from tryptophan
Inc Cl - in sweat
50. bilateral - massive enlargement of of kidneys due to multiple large cysts
CFTR gene - 7 - Phe 508
Adds 2 carbon with the help of biotin
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
ADPKD
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