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Test your basic knowledge |
Biochemistry
Start Test
Study First
Subjects
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health-sciences
,
science
Instructions:
Answer 50 questions in 15 minutes.
If you are not ready to take this test, you can
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. Name as many x- linked recessive disorders as you can
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2. Describe robertsonian translocation
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
Pyruvate carboxylase - PEP carboxykinase - fructose 1 -6 biphosphatase - glucose 6 phosphatase
Nonreciprocal chromosomal translocation that commonly involves chromosome pairs 13 - 14 - 15 - 21 - 22
Mental retardation - growth retardation - seizures - fair skin - eczema - musty body odor
3. What does a mischarge tRNA do
Alpha 1 -4 glucosidase
Cheilosis - inflammation of lips - scaling and fissures at the corner of the mouth - corneal vascularization
Breaks down acyl - coa to acetyl coa groups in mito
Reads usual codon but inserts wrong AA
4. What is the energy source for translocation
Alpha1 antitrypsin
GTP
Enter G1 from G0 when stimulated - hepatocytes and lymphocytes
RNA poly II
5. What is the source of energy in the fasting state between meals
B6
Hepatic glycogenolysis >> hepatic gluconeogenesis > adipose release of FFA
Inhibits RNA polymerase II - found in death cap mushrooms
Adds an inorganic phosphate onto substrate without using ATP
6. What apolipoprotiens are on VLDL
Coarse facial features - clouded corneas - restricted joint movements - high plasma levels of lysosomal enzymes - often fatal in childhood
B-100 - CII and E
SnRNPs and other proteins
Terminal regions - tropocollagen
7. FAP is due to deletion On what gene On what chromosome
Coenzyme A - lipoamide
APC on chromosome 5
Reads usual codon but inserts wrong AA
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
8. Patients with chronic granulomatous disease are at increased risk of infection From which kind of organisms
75 to 90 - cloverleaf
2pq
Catalase positive (catalase neg produce H2O2 the cell can use) like S. aureus or aspergillus
Antibiotic use or excessive ingestion of raw eggs
9. How many ATP are created by aerobic metobolism of glucose in muslce via what shuttle
30 - glycerol -3- phosphate shuttle
Collagen
Assistance of upper extremities to stand up
Phosphenolpyruvate to pyruvate catalyzed by pyruvate kinase
10. What does desmin stain for
One
Acetyl - CoA
Von Gierke's - Pompe - Cori - McArdle
Muscle
11. In which single strand DNA repair mechanism do specific endonucleases release the oligonucTIDE containing bases and DNA poly and ligase fill and reseal - and what disease occures when this is mutated
Nucleotide excision repair - xeroderma pigmentosum; unable to repair thymidine dimers caused by UV light
Glucose -6 phosphate
Glycolysis and the glycogen synthesis in the liver - hexokinase or glucokinase
Neg to pos
12. What two pathways is carbomoyl phosphate involved in - and what enzyme deficiency leads to a build of carbamoyl phosphate leading to what element in pyrimidine synthesis
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
Scrutinizes - hydrolyzes the bond
Dermatitis - alopecia - enteritis
Mutations at different loci can produce the same phenotype - marfans - MEN 2B - homocystinuria; all cause marfinoid habitus - also albinism
13. What is NADPH used for
Anabolic processes as a supply of reducing equivalents
Vit K antagonist
Semiconservative - continuous and discontinuous strands (okazaki fragments)
LPL def - or altered apoCII - elevated TG and cholesterol - causes pancreatitis - hepatosplenomegaly and eruptive/pruritic xannthomas - no risk of atherosclerosis
14. What causes B12 def
Marfans
Macrocytic - megaloblastic anemia - no neurologic sx - most common vit def in the US - seen in EtOH and pregs
Malabsorption (sprue - enteritis) lack of intrinsic factor from pernicious anemia - gastric bypass surgery or absence of terminal ileum as in Crohns
1- capping on 5' (7- methyguanosine) 2 - polyadenylation on 3' end 3 - splicing out of introns
15. What is the activated carrier for phosphoryl
ATP
Colchicine
Meiotic nondisjunction of homologous chromosomes (associated with advanced maternal age) leading to trisomy 21
Lariat shape in order and remove intron precisely and join 2 exons
16. What is the TX for pyruvate dehydrogenase deficiency
Inc intake of ketogenic nutrients - high in fact content or inc lysine or leucine
Makes RNA primer on which DNA poly III can initiate replication
Terminal regions - tropocollagen
B48 - AIV - CII - E
17. What is kartageners syndrome
Immotile cilia due to dynein arm defect - male and femail infertile - bronchiectasis - recurrent sinusitis - associated with situs inversus
2 pyruvate - 2ATP - 2NADH - 2H+ - 2H2O
Inc orotic acid in urine - megaloblastic anemia (does not correct with B12 or folate) - failure to thrive
In ER - glucose 6- P to glucose
18. Milder form of type I with nl blood lactate levels - dz and enzyme
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19. What does a northern blot use as its sample
RNA
Inability to transport LCFAs into mitochondria results in toxic accumulation causing weakness - hypotonia - hyperketotic hyperglycemia
In mitochondria - pyruvate to oxaloacetate - requires biotin - ATP - activated by acetyl coA
Polycystic liver disease - berry aneurysm - mitral valve prolapse
20. What is a missense mutation
Dec phenylalanine (contained in aspartame - Nutrasweet) inc tyrosine in diet
Changed AA (convservative - new AA is similar in chemical structure)
Debranching enzyme
Removes phosphate group from substrate
21. What does fomepizole do
Inhibits EtOH dehydrogenase and is an antidote for methanol and ethylene glycol poisoning
Hepatic glycogenolysis >> hepatic gluconeogenesis > adipose release of FFA
Wobble
Glycine - aspartate - glutamine
22. What is the RDE of gluconeogenesis
Neural tube
Fructose 1 -6 bisphosphate
Stop codon is recognized by release factor - and completed protein is released from ribosome
Breaks down acyl - coa to acetyl coa groups in mito
23. What is NADPH's role inside RBCs
3 NADH - 1 FADH2 - 2 CO2 - 1 GTP = 12 ATP (x2 per glucose)
Keep glutathione reduced so it can detoxify free radicals and peroxides
AMP - fructose 2 -6 BP
Constitutive - random insertion of gene into mouse genome and conditional - targeted insertion or deletion of gene through homologous recombination with mouse gene
24. Wgat substances directly inhibit mitochondrial ATPase - causing an inc in proton gradient - no ATP because pump is stopped
Free ribosomes
Glycogen synthase
Stored ATP - creatine phosphate - anaerobic glycolysis
Oligomycin
25. Describe the pathophys of the aorta in a pt with marfans - and the eyes
5' of the incoming nucleotide
Differences in phenotype depend on whether the mutation is of maternal or paternal origin - prader - willi and angelman's syndrome
4 under the floor
Cystic medial necrosis of aorta leading to aortic incompetence and dissecting aortic aneurysms - floppy mitral valve - subluxation of lenses
26. What does GFAP stain for
L form
Asp and Glu
Glucose -6 phosphate
Neuralgia
27. Of the four possible fates for pyruvate - which one can replenish TCA cycle or be used in gluconeogenesis
B6
Oxalacetate
HGPRT - defective purine salvage - excess uric acid production
N to C
28. How does patients present with ADPKD
Q -
Pyruvate dehydrogenase - ATP - NADH - acetyl - CoA
Type II
Flank pain - hematuria - HTN - progressive renal failure
29. Which enzyme involved in RNA synthesis does not require a template
Isocitrate dehydrogenase
Lack of tyrosinase (no melanin) AR ordefective tyrosine transporters (less tyrosine) - can results in lack of migration of NC cells
Offspring receives 2 copies of a chromosome from 1 parent and no copies from the other parent
Poly A polymerase - signal is AAUAA
30. What co - factors are required for the pyruvated dehydrogenase complex
Pyrophosphate (B1 - thiamine - TPP) FAD (B2 - riboflavin) - NAD (B3 - niacin) - CoA (B5 pantothenate) - lipoic acid
In cytosol - oxaloacetate to phosphenolpyruvate - requires GTP
Connective tissue
Secretion of abnl thick mucus that plugs lungs - pancreas - liver
31. Which antifungal drugs work on microtubules
Coarse facial features - clouded corneas - restricted joint movements - high plasma levels of lysosomal enzymes - often fatal in childhood
Griseofulvin
Basement membrane or basal lamina
Disease
32. What are the products for glycolysis
Acetly- CoA - CO2 - NADH
2 pyruvate - 2ATP - 2NADH - 2H+ - 2H2O
Lack of proper dietary therapy during pregnancy leading to microcephaly - mental retardation growth retardation - congenital heart defects
Terminal regions - tropocollagen
33. What does NADPH oxidase deficiency result in and why
Chronic granulomatous disease - no respiatory burst - no formatino of ROS
Pyruvate - NAD+ - CoA
TRNA
Fasting
34. Where do you find elastin and What does it do
Enhancers
Adenosine to inosine
Phosphenolpyruvate to pyruvate catalyzed by pyruvate kinase
Lungs - large arteries - elastic ligaments - vocal cords - ligamenta flava - stretch.
35. Synthesis of vit B3 requires what other vitamin
Progressive hreditary nephritis and deafness - associated with occular disturbances - due to abnl type IV collagen - usually x- linked recessive
Malabsorption and steatorrhea (ADEK)
VMA
B6
36. What is the hardy weinber heterozygote prevalence
Nonreciprocal chromosomal translocation that commonly involves chromosome pairs 13 - 14 - 15 - 21 - 22
Liver and leafy veggies
2pq
More than 1 codon may code for the same amino acid
37. Which RNA poly opens DNA at promotor site
Tay- Sachs - hexosaminidase A - GM2 ganglioside - AR
RNA poly II
Neurofibromatosis type 1 (von Recklinghausens disease)
Convulsions - hyperirritability - peripheral neuropathy - deficiency inducible by INH and oral contraceptives - sideroblastic anemais
38. What is the energy source for tRNA actication (charging)
Sulfation
Peroxide
Microtubules
ATP
39. What are cyclin - CDK complexes
Nonreciprocal chromosomal translocation that commonly involves chromosome pairs 13 - 14 - 15 - 21 - 22
F16BP
Must be both activated and inactivated for cell cycle to progress
Lysine and arginine
40. metaphse chromosomes are stained - ordered and numbered according to morphology - size - arm length ratio - banding pattern
Depletes alpha - ketoglutarate leading to inhibition of TCA cycle - tremor slurring of speech - somnolence - vomiting - cerebral edema - blurring of vision
Never go to G0 - divide rapidly with a short G1 - bone marrow - gut epi - skin - hair follicles
Glucose - 2Pi - 2ADP - 2NAD+
Karyotyping
41. 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
Fatty acid oxidation - acetyl - Coa production - TCA cycle - OXPHOS
RRNA
Basement membrane or basal lamina
42. Adenosine deaminase deficiency is an important cause of what immunodeficiency?
AR
Dec DNA - dec lymphos leads to SCID
Inc melt - dec fluidity
Delayed wound healing - hypogonadism - dec in adult hair - dysguesia - anosmia - may predispose to EtOH cirrhosis
43. What is the amino acid precuror for creatine - urea and nitric oxide
Niacin - constituent of NAD and NADP - derived from tryptophan
Alpha 1 -4 glucosidase
Arginine
NF2 on chromosome 22
44. characterize autosomal recessive inheritance
Glycogenolysis to form glucose
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
2 rings
Neonatal hemorrhage with inc PT and PTT - but nl bleeding time - can also occur after prolonged use of broad spectrum Abx
45. peripheral neuropathy - developmental delay - optic atrophy - glopoid cells
O- oligosaccharaides
Liver - also in kidney and gut epithelium
Krabbes - galactocerebrosidase - galactocerebroside - AR
Microtubules
46. What is the RDE of ketogenesis
LDL
HMG- CoA synthase
Diphyllobothrium latum
Acetoacetate and beta hydroxybutyrate
47. central and peripheral demyelination with ataxia and dementia
Karyotyping
Metachromatic leukodystrophy - arylsulfatase A - cerebroside sulfate AR
2 -4 DNP - aspirin
P+q = 1
48. What causes biotin def
Antibiotic use or excessive ingestion of raw eggs
Only processed RNA
Neither of 2 alleles is dominant - blood groups
Phosphenolpyruvate to pyruvate catalyzed by pyruvate kinase
49. How are the many staggered tropocollagen molecules reinforced
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
Covalent cross - linking by lysyl oxidase to make collagen fibrils
Glycogen synthase
Hereditary spherocytosis
50. What are the 3 AR forms of homocystinuria
SnRNPs
Site of steroid synthesis and detoxification of drugs and poisons
Stored and undergoes glycogenolysis to maintain blood sugar at appropriate levels
Cystathionine synthase def - dec affinity of cystathionine synthase for pyridoxal phosphate - homocystein methyltransferase def