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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
.
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 breakdown product of epi
Cheilosis - inflammation of lips - scaling and fissures at the corner of the mouth - corneal vascularization
Metanephrine
Never go to G0 - divide rapidly with a short G1 - bone marrow - gut epi - skin - hair follicles
Free ribosomes
2. energy malnutrition resulting in tissues and muscle wasting - loss of subQ fat - variable edema
CAG
Fabrys - alpha galactosidase A - ceramide trihexoside - XR
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
Marasmus - muscle wasting
3. What does Ehlers Danlos cause and why
Faulty collagen synthesis - hyperextensible skin - tendency to bleed - easy bruising - hypermobile joints
MEN - 2A and 2B with ret gene
Site of steroid synthesis and detoxification of drugs and poisons
Cyclin dependent kinases;constitutive and inactive
4. phosphorylation of glucose to yield glucose 6 phosphate serves as the 1st step of what rxns and what enzymes catalyze it
Glycogenolysis to form glucose
Rickets in kids - bending bones - osteomalacia in adults - hypocalcemia tetany - breast milk has dec vit D (supplement in dark skinned patients)
Glycolysis and the glycogen synthesis in the liver - hexokinase or glucokinase
Lysine and arginine
5. What is the main source of folate
Stop codon is recognized by release factor - and completed protein is released from ribosome
Foliage - small reserve in liver - eat green leaves
Sucrose = glucose + fructose - lactose = glucose + galactose
Brittle bone disease - most commonly lead AD with abnl type I collagen causing multiple fractures with minimal trauma - blue sclerae due to translucency of connective tissue over the choroid - hearing loss - dental imperfections due to lack of dentin
6. What does acetyl - CoA become before becoming palmitate
UGA - UAA and UAG
Malonyl coa (+ biotin= palmitiate - 1 16C fatty acid)
Chronic granulomatous disease - no respiatory burst - no formatino of ROS
Ubiquitous - low Km - high affinity - low capacity low Vmax - uninduced by insulin
7. What apolipoproteins are on chylomicrons
Miscarriage - stillbirth - chromosomal imbalance (down - patau)
HMP shunt
Hypoglycemia
B48 - AIV - CII - E
8. congenital microdeletion of long arm of chromosoe 7 with findings of elfin facies - mental retardation - hypercalcemia - well developed verbal skills - extreme friendliness with strangers - cardiovascular problems
Progressive hreditary nephritis and deafness - associated with occular disturbances - due to abnl type IV collagen - usually x- linked recessive
Williams syndrome
Carbomoyl phosphate synthetase II
Purines= A - G pyrimidine = C - T (U)
9. What happens on the oxidative arm of the HMP shunt and What is the key enzyme
NAD+
Autosomal recessive diseases
Enhancers
Glucose 6P to CO2 - 2NADPH - ribulose 5P - G6PD - rate limiting step
10. Which kind of osteogenesis imperfecta is fata in utero or in neonatal period
25OHD3
Type II
Transfers cholesterol from mature HDL to VLDL - IDL and LDL (cholesterol ester transfer protein)
Lysine and leucine
11. What does a pyruvate dehydrogenase deficiency lead to and What are the findings
Degredation of dietary TG in small intestine
Backup of substrate (pyruvate and alanine) resulting in lactic acidosis - congenital or acquired from thiamine def in EtOH - neuro defects
Transmitted through both parents - affected mother may have affected children - affected father will have affected children
CAG
12. What are bite cells and when do you see them
HMP shunt
Result from phagocytic removal of heinz bodies my macs - G6PD def
Dec phenylalanine (contained in aspartame - Nutrasweet) inc tyrosine in diet
O- oligosaccharaides
13. What liberates glucose from glucose 6 P
Changed AA (convservative - new AA is similar in chemical structure)
Excess NADH shunts oxaloacetate to malate (backwards) stalling the TCA and shunting glucose and FFA to ketone body production
Glucose 6 phosphatase
X linked frame shif mutation
14. What does hepatic TG lipase do
Hypoglycemia
Degradation of TG remaining in IDL
Glucose - 2Pi - 2ADP - 2NAD+
IMP precursor
15. What substances inhibit phosphofructokinase -1
B100
Glucose - 2Pi - 2ADP - 2NAD+
Inhibits lipoic acid - vomiting - rice water stools - garlic breath
ATP - citrate
16. What is proteasomal degredation
Attachment of ubiquitin to defective proteins tag them for breakdown
Read from a fixed starting point as a continuous sequence of bases
Von gierkes - glucose 6 phosphatase
Nonsense > missense > silent
17. What is linkage disequilibrium
Phenytoin - MTX - and sulfonamides
Kidney - ears - eyes
Tendency for certain alleles at 2 linked loci to occur together more often than expected by chance - varies is different populations
In mitochondria - pyruvate to oxaloacetate - requires biotin - ATP - activated by acetyl coA
18. Where do you find elastin and What does it do
Lungs - large arteries - elastic ligaments - vocal cords - ligamenta flava - stretch.
Ribos first then deoxyribos with ribonucleotide reductase
2 -4 DNP - aspirin
Inc insulin - dec cAMP - dec PKA
19. What does apoA 1 do
Propionyl - CoA to methylmalonyl - CoA (3C to 4C)
2 pyruvate - 2ATP - 2NADH - 2H+ - 2H2O
Glycogenolysis to form glucose
Activates LCAT
20. What are the findings in Lesch - Nyhan
Blood - bone marrown - amniotic fluid - placental tissue
Muscle
Alpha - ketoglutarate dehydrogenase complex
Retardation - self mutilation - aggression - hyperuricemia - gout - choreoathetosis
21. What are the two transgenic strategies in mice
In ER - glucose 6- P to glucose
Phenylethamolamine N methyl transferase
Constitutive - random insertion of gene into mouse genome and conditional - targeted insertion or deletion of gene through homologous recombination with mouse gene
Leu - lys
22. What are the names and sources of the two types of vit D found in nature
Animal products - synthesized only by microorganisms - large reserve pool - mainly in liver
D2 = ergocalciferol - ingested from plants D3 = cholecalciferol - ingested from milk - formed in sun exposed skin
Aldolase B - AR - fructose 1P accumulates causing a dec in availabel phosphate which inhibts glycogenolysis and gluconeogenesis leading to hypoglycemia - cirrhosis - jaundice and vomiting
Ornithin transcarbamoylase def - x linked recesssive - other urea cycle enzymes defs are autosommal recessive
23. fibrillin defect leading to connective tissue disorder affecting skeleton - heart - and eyes
Glucose 6 phosphatase
Elastase - inhibited by alpha1 antitrypsin
Marfans
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
24. What other inflammatory process can induce a hemolytic anemia in NADPH defieicnt patients
Adenosine to inosine
Tuberous sclerosis
Alpha - ketoglutarate dehydrogenase complex
Infection - free radicals generated by inflammatory response
25. Name as many x- linked recessive disorders as you can
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26. What apolipoprotein is on LDL
HMG- CoA synthase
If pt inherits or develops a mutation in a tumor suppresor gene - the complementary allele must be deleted/mutated before cancer develops - not true of oncogenes - retinoblastoma
B100
2 -4 DNP - aspirin
27. what disease can cause pellagra
Hartnup disease - dec tryptophan absoprtion - malignant carcinoid syndrome (inc tryptopha metabolism) and INH (dec vit B6)
HMG- CoA (HMG- CoA to mevalonate
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
Cheilosis - inflammation of lips - scaling and fissures at the corner of the mouth - corneal vascularization
28. What residues are hydroxylated in step 2 of collagen synthesis - and What does this require
Proline and lysine - vit C
Inc orotic acid in urine - megaloblastic anemia (does not correct with B12 or folate) - failure to thrive
Riboflavin - cofactor in oxidation and reduction (FAD and FMN are derived from riboflavin)
Via 1 proprionyl - CoA which can enter the TCA as succinyl - CoA and undergo gluconeogenesis
29. In the chromatin structure - which histones form the octamer - and which ties the nucleosome beads together in a string?
Unmethylated - newly synthesized - HNPCC
More than 1 codon may code for the same amino acid
Athralgias - fatigue - headaches - skin changes - sore throat - alopecia
Octamer = 2 sets of H2A - H2B - H3 - H4 - tie =H1
30. Which aspect of the spliceosome do patients with lupus make antibodies against
Epithelial cells
Specific glycosylases - AP endonuclease
SnRNPs
Diphyllobothrium latum
31. What is the activated carrier for phosphoryl
RRNA
B12 and folate
B-100 - CII and E
ATP
32. What do the single stranded binding proteins do
Hepatic overproduction of VLDL causing pancreatitis - elvelated TGs and VLDL
Prevent strands from reannealing
Impaired glucose breakdown leading to ATP depletion; highly affected aerobic tissues like brain and heart affected first
Citrate - isocitrate - alpha ketoglutarate - succinyl - CoA - succinate - fumarate - malate - oxaleoacetate
33. What happens in a B12 def
Alpha1 antitrypsin
Phosphenolpyruvate to pyruvate catalyzed by pyruvate kinase
Macrocytic - megaloblastic anemia - hypersegmented PMNs - neurologic symptoms - (parasthesias - subacute combined degeneration) due to abnl myelin - prolonged becomes irreversible
Paclitaxel
34. Fluorescent DNA or RNA probe binds to specific gene of interest - used for specific localization of genes and direct visualization of anomalies - like microdeletions - at molecular level
Glycogen phosphorylase
FISH
Genetic code is conserved throughout evolution - except for mito - archaebacteria - mycoplasma - and some yeast
Protein
35. 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
Sulfation
Regulatory proteins that control cell cycle events; phase specific; activate CDKs
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
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
36. Who typically has lactase def
Pyrophosphate (B1 - thiamine - TPP) FAD (B2 - riboflavin) - NAD (B3 - niacin) - CoA (B5 pantothenate) - lipoic acid
Cori's - debranching enzyme alpha 1 -6 glucosidase - gluconeogenesis intact
African Americans and Asians
Liver and beta cells of pancreas - low affinity - high Km - high capacity - high Vmax - induced by insulin
37. What is the name is fxn of vit B3
Malabsorption and steatorrhea (ADEK)
Bone - skin - tendon - dentin - fascia - cornea - late wound repair
Debranching enzyme
Niacin - constituent of NAD and NADP - derived from tryptophan
38. What does an umabiguous genetic code refer to...
Each codon specifies only 1 amino acid
CAG
Dopamine beta hydroxylase
CAG - 4
39. What are the symptoms of vit A excess
Athralgias - fatigue - headaches - skin changes - sore throat - alopecia
EtOH dehydrogenase and acetaldehyde dehydrogenase
Excess ATP - dATP - feeback inhibits ribonucleotide reductase - no DNA synthesis
Inc melt - dec fluidity
40. What does glycosylation of pro alpha chian yield and What is the structure
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
Procollagen - triple helix of 3 alpha collagen chains
Cleft palate - cardiac abnl - pregs test
Inhibits RNA polymerase II - found in death cap mushrooms
41. What are the results of CF on male fertility
Males are infertile due to bilateral absence of vas deferens
30 - 50 - 70
Protein
Dec AFP - inc betahCGH - dec estradiol - inc inhibin - inc nuchal translucency on US
42. What is RNAi used for
In cytosol - oxaloacetate to phosphenolpyruvate - requires GTP
DsRNA promotes degradation of target mRNA knocking down gene expression
Changed AA (convservative - new AA is similar in chemical structure)
Huntingtons
43. What reaction does adenosine deaminase normally catalyze
Lungs - large arteries - elastic ligaments - vocal cords - ligamenta flava - stretch.
Adenosine to inosine
Mitochondria
1 ring
44. How does warfarin work
No mutation occuring at the locus - no selection for any genotypes at the locus - completely random mating - no migration
Lebers hereditary optic neuropathy - degeneration of retinal ganglion cells and axons - leads to acute loss of central vision. Mitochondrial myopathies
3 NADH - 1 FADH2 - 2 CO2 - 1 GTP = 12 ATP (x2 per glucose)
Vit K antagonist
45. In eukaryotes - What does RNA poly III make
G6PD
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
Must be both activated and inactivated for cell cycle to progress
TRNA
46. What is sorbitol - how and why is it made
Paclitaxel
Tryptophan excretion in urine and dec absorption from the gut leading to pellagra
Plasma membrane - 3Na out - 2K in - ATP on cytoplasmic side
Alcohol version of glucose - can trap glucose in cell - aldose reductase
47. What is the TX for pyruvate dehydrogenase deficiency
Cystic medial necrosis of aorta leading to aortic incompetence and dissecting aortic aneurysms - floppy mitral valve - subluxation of lenses
Inc intake of ketogenic nutrients - high in fact content or inc lysine or leucine
Intermediate filaments
Dermatitis - alopecia - enteritis
48. What two proteins make up microtubules and how are they arranged
Read from a fixed starting point as a continuous sequence of bases
Seals.
Alpha and beta tubulin - dimers have two GTP bound
Orotic acid to UMP
49. How do labile celss grow and regenerate and What are examples
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
Each codon specifies only 1 amino acid
Myeloperoxidase
Never go to G0 - divide rapidly with a short G1 - bone marrow - gut epi - skin - hair follicles
50. What 3 syndromes are associated with vit B1 def
Protein
Wernicke - korsakoff - dry and wet beriberi
Pyruvate - NAD+ - CoA
Gamma carboxylation of glutamic acid residues on various proteins concerned with blood clotting - synthesized in intestinal flora