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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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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. in a 100 meter sprint Where does energy come from
LCAT (lecithin cholesterol acyltransferase)
Chylomicrons
TTP
Stored ATP - creatine phosphate - anaerobic glycolysis
2. What is the amino acid precursor for GABA and glutathione
Liver - ovaries - seminal vesicles
More than 1 codon may code for the same amino acid
Alpha 1 -6 and alpha 1 -4
Glutamate
3. What enzyme degrades a small amount of glycogen in lysosomes
Comlex II
Alpha 1 -4 glucosidase
Isocitrate dehydrogenase
Depletes alpha - ketoglutarate leading to inhibition of TCA cycle - tremor slurring of speech - somnolence - vomiting - cerebral edema - blurring of vision
4. What is the TX for PKU
Breaks down acyl - coa to acetyl coa groups in mito
RRNA
Dec phenylalanine (contained in aspartame - Nutrasweet) inc tyrosine in diet
4 under the floor
5. What are CDKs
Cyclin dependent kinases;constitutive and inactive
Inc insulin - dec cAMP - dec PKA
Conversion of NE to epi
Phenylalanine hydroxylase
6. In mismatch repair - which strand is recognized and In what hereditary syndrome is this defective
Two
Degredation of dietary TG in small intestine
RRNA
Unmethylated - newly synthesized - HNPCC
7. What metabolic rxns occur in both the cytoplasm and mitochondria
Lysine and leucine
One
Heme synthesis - urea cycle and gluconeogenesis - HUGs take two
Intermediate filaments
8. What are the findings in Down's syndrome
Tryosine hydroxylase
Gamma carboxylation of glutamic acid residues on various proteins concerned with blood clotting - synthesized in intestinal flora
Ornithin transcarbamoylase def - x linked recesssive - other urea cycle enzymes defs are autosommal recessive
Mental retardation - flat facies - prominent epicanthal folds - simian crease - gap between 1st 2 toes - duodenal atresia - congenital heart disease (ASD) - inc risk of All and Alzheimers
9. spheroid RBCs due to spectrin or ankyrin defect - hemolytic anemia - inc MHCH - splenectomy is curative
Hereditary spherocytosis
Dermatitis - enteritis - alopecia - adrenal insuff
Poor defense from oxidizing agents - fava beans - sulfonamides - primaquine - antituberclosis drugs leadig to hemolytic anemia.
Glycolysis - fatty acid synthesis - HMP shunt - protein synthesis (RER) - steroid synthesis (SER)
10. What does GFAP stain for
Ribose 5- P to PRPP
Neuralgia
NADPH
Malabsorption and steatorrhea (ADEK)
11. How does patients present with ADPKD
Silencers
Blood - bone marrown - amniotic fluid - placental tissue
Flank pain - hematuria - HTN - progressive renal failure
Dec phenylalanine (contained in aspartame - Nutrasweet) inc tyrosine in diet
12. What are the clinical features of I cell diesase
Coarse facial features - clouded corneas - restricted joint movements - high plasma levels of lysosomal enzymes - often fatal in childhood
Debranching enzyme
Tyrosine
B48 - AIV - CII - E
13. What causes Hartnup's disease
Remain in G0 - regenerate from stem cells - neurons - skeletal/cardiac muscle - RBCs
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
Glycine
AR disorder characterized by defective neutral amino acid transporte on renal and intestinal epith cells
14. What is Gowers maneuver
Microarrays
Assistance of upper extremities to stand up
Pyruvate to oxaloacetate (3C to 4C)
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
15. benign asymptomatic condition with elevated levels of fructose in urine and blood - dz and enzyme
Hunters - iduronate sulfatase - heparan sulfate/dermatan sulfate - XR
Hypoglycemia
Essential fructosuria - fructokinase AR
For proteins and lipids from ER to plasma membrane - lysosomes and secretory vesicles
16. Where is vit A found in the diet
Liver and leafy veggies
Glucose to G-6P - hexokinase and fructose -6P to fructose 1 -6 BP phosphofructokinase -1
Anchor muscle fibers - primarily in skeletal and cardiac muscle
APRT + PRPP
17. 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
Differences in phenotype depend on whether the mutation is of maternal or paternal origin - prader - willi and angelman's syndrome
Mcardle's - skeletal muscle glycogen posphorylase
One
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
18. Gene imprinting implies that How many alleles are active at a single locus
Chronic granulomatous disease - no respiatory burst - no formatino of ROS
Targets the proteins for lysosome
One
Acetoacetate and beta hydroxybutyrate
19. delivers hepatic TGs to peripheral tissue - secreted by liver
Niacin - constituent of NAD and NADP - derived from tryptophan
Cobalamin - cofactor of homocysteine and methyltransferase - methylmalonyl CoA mutase
Liver and beta cells of pancreas - low affinity - high Km - high capacity - high Vmax - induced by insulin
VLDL
20. What is the physiologic role of dystrophin
Glutamine PRPP amidotransferase
Anchor muscle fibers - primarily in skeletal and cardiac muscle
Ca/calmodulin in muscle to coordinate with muscle activity
Chronic granulomatous disease - no respiatory burst - no formatino of ROS
21. What does cytokeratin stain for
Epithelial cells
Neg to pos
Oxidative is irreversible
Malabsorption (sprue - enteritis) lack of intrinsic factor from pernicious anemia - gastric bypass surgery or absence of terminal ileum as in Crohns
22. facial lesions - hypopigmented 'ash leaf spots -' cortical and retinal hamartomas - seizures - mental retardation - renal cysts - renal angiomyolipomas - cardiac rhabdomyomas - inc risk of astrocytomas
Never go to G0 - divide rapidly with a short G1 - bone marrow - gut epi - skin - hair follicles
Arg and his inc in histones Which bind negatively charged DNA
Tuberous sclerosis
Gaucher's - beta glucocerebrosidase - glucocerebrosie - AR
23. How do glucagon/epi lead to glycogenolysis
Enter G1 from G0 when stimulated - hepatocytes and lymphocytes
Consesus sequenec of base pairs
Hypercalciuria - loss of appetite - stupor - seen in sarcoidosis from activation fo vit D by epithelioid macrophage
Adenylyl cycle - inc cAMP - inc PKA - glycogen phosphorylase kinase activated - glycogen phosphoylase active - glycolysis
24. What enzyme adds Cl - to the H202 to makes bleach
IMP precursor
Backup of substrate (pyruvate and alanine) resulting in lactic acidosis - congenital or acquired from thiamine def in EtOH - neuro defects
Meiotic nondisjunction of homologous chromosomes (associated with advanced maternal age) leading to trisomy 21
Myeloperoxidase
25. What is the RDE of fatty acid synthesis
Lysine and arginine
Acetyl - CoA carboxylase (ACC)
Pseudomonas and s aureus
Cystine kidney stones - cystine staghorn calculi - cystine is made of two cysteines connected by disulfide bond
26. cell signaling defect of fibroblast growth factor (FGF) receptor 3 - results in dwarfism - short limbs - head/trunk nl size - associated with advanced paternal age
Achondroplasia
Removal of N or C termal propeptides from zymogens to generate mature proteins
Microtubules
Anabolic processes as a supply of reducing equivalents
27. Where is vit B12 found
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
30 - glycerol -3- phosphate shuttle
ATP - citrate
Animal products - synthesized only by microorganisms - large reserve pool - mainly in liver
28. What is the amino acid precursor for catecholamines
In cytosol - oxaloacetate to phosphenolpyruvate - requires GTP
Modifies N- oligosaccharides
Phenylalanine
Phenylketones in urine
29. fibrillin defect leading to connective tissue disorder affecting skeleton - heart - and eyes
Carbomoyl phosphate synthetase I
Offspring receives 2 copies of a chromosome from 1 parent and no copies from the other parent
Marfans
FISH
30. Why is G6PD def more common among patients of african decent
Protective against malaria
X linked frame shif mutation
Fabrys - alpha galactosidase A - ceramide trihexoside - XR
Active secretion in lungs and GI - reabsorbs in skin
31. What happens in hyperammonemia
Animal products - synthesized only by microorganisms - large reserve pool - mainly in liver
Alpha 1 -4 glucosidase
Protein kinase A
Depletes alpha - ketoglutarate leading to inhibition of TCA cycle - tremor slurring of speech - somnolence - vomiting - cerebral edema - blurring of vision
32. Type II collagen
Intermediate filaments
Mental retardation - hyperphagia - obesity - hypogonadism - hypotonia - deletion of normally active paternal allele on chromosome 15
Glucose - 2Pi - 2ADP - 2NAD+
Cartilage - hyaline - vitreous body - nucleus pulposus
33. What defects characterize DiGeorge syndrome
Thymic - parathyroid and cardiac
Hereditary defect of renal tubular amino acid transporter for cystein - ornithine - lysine and arginine in PCT
Defect in fibrillin
Pseudomonas and s aureus
34. Which end of the tRNA is the amino acid bound to...
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35. What amino acid makes up most of the octamer
Miscarriage - stillbirth - chromosomal imbalance (down - patau)
Propionyl - CoA to methylmalonyl - CoA (3C to 4C)
Lysine and arginine
Animal products - synthesized only by microorganisms - large reserve pool - mainly in liver
36. What is the RDE of TCA cycle
Inability to transport LCFAs into mitochondria results in toxic accumulation causing weakness - hypotonia - hyperketotic hyperglycemia
BOne
Isocitrate dehydrogenase
Inhibits acetaldehyde dehydrogenase and contributes to hangover symptoms
37. What does PURe As Gold and CUT The Py stand for
Purines= A - G pyrimidine = C - T (U)
Protein kinase A
Phenylalanine
N- acteylcysteine - cleave disulfide bonds within mucus glycoproteins
38. What is the treatment for orotic aciduria
Activates LCAT
Inc glucagon - inc cAMP - inc PKA
Oral uridine administration
Avidin
39. What is the RDE of gluconeogenesis
30 - glycerol -3- phosphate shuttle
NF2 on chromosome 22
Fructose 1 -6 bisphosphate
Octamer = 2 sets of H2A - H2B - H3 - H4 - tie =H1
40. What apolipoproteins are on IDL
B100 and E
Glycogen and FFA oxidation; glucose conserved for final sprinting
5' of the incoming nucleotide
Promotor - TATA box - and CAAT box - AT rich
41. What are the 4 assumption of the Hardy Weinberg law
Binds 50S - blocking translocation
Acetoacetate and beta hydroxybutyrate
Liver hepatocytes and steroid producing cells of the adrenal cortex
No mutation occuring at the locus - no selection for any genotypes at the locus - completely random mating - no migration
42. What does a pyruvate dehydrogenase deficiency lead to and What are the findings
Inhibits EtOH dehydrogenase and is an antidote for methanol and ethylene glycol poisoning
Ribose 5- P to PRPP
Mutations at different loci can produce the same phenotype - marfans - MEN 2B - homocystinuria; all cause marfinoid habitus - also albinism
Backup of substrate (pyruvate and alanine) resulting in lactic acidosis - congenital or acquired from thiamine def in EtOH - neuro defects
43. What substance in egg whites binds biotin
Aberrant 3rd and 4th branchial arches causing 22q11 deletion - cleft palate - abnl facies - thymic dysplasia - cardiac defects - hypocalcemia secondary to parathyroid aplasia
Lariat shape in order and remove intron precisely and join 2 exons
Avidin
Citrate - acetyl coa from mito to cyto
44. What are the teratogenic effects of vit A and What test must be ordered before starting a woman on isotreitinoin for severe acne
SNP
Locus heterogeneity - ocular albinism is x- linked recessive
Cleft palate - cardiac abnl - pregs test
Tendency for certain alleles at 2 linked loci to occur together more often than expected by chance - varies is different populations
45. What substances inhibit phosphofructokinase -1
Prokaryotic only - elongates leading strand by adding deoxnucTIDE to the 3' end. Elongates lagging strand until it reaches primer of preceeding fragment. 3' to 5' exonuclease activity 'proofreads' each added nucTIDE
ATP - citrate
Locus heterogeneity - ocular albinism is x- linked recessive
After day 1
46. What is the energy source for tRNA actication (charging)
ATP
2pq
Tryptophan
Severity of disease worsens or age of onset of disease is earlier in succeeding generations - huntingtons
47. What is the composition of urea and where do each part derive from
SnRNPs
NH2-(C=O) - NH2 one NH2 from ammonia the C=O from CO2 - and the other NH2 from aspartate
Lungs - large arteries - elastic ligaments - vocal cords - ligamenta flava - stretch.
Glycine
48. What happens in zinc def
Proline and lysine - vit C
N to C
Heme synthesis - urea cycle and gluconeogenesis - HUGs take two
Delayed wound healing - hypogonadism - dec in adult hair - dysguesia - anosmia - may predispose to EtOH cirrhosis
49. phosphorylation of glucose to yield glucose 6 phosphate serves as the 1st step of what rxns and what enzymes catalyze it
RRNA
Glycolysis and the glycogen synthesis in the liver - hexokinase or glucokinase
Glycine
Glycogen synthase
50. What converts tyrosine to DOPA
Tryosine hydroxylase
Essential fructosuria - fructokinase AR
HGPRT - defective purine salvage - excess uric acid production
Secretion of abnl thick mucus that plugs lungs - pancreas - liver
Sorry!:) No result found.
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