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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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1. cardiomegaly - systemic findings leading to early death - dz and enzyme

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2. What are covalent alterations
Metachromatic leukodystrophy - arylsulfatase A - cerebroside sulfate AR
Essential fructosuria - fructokinase AR
Phosphorylation - glycosylation - hydroxylation
Excess ATP - dATP - feeback inhibits ribonucleotide reductase - no DNA synthesis

3. What is disulfiram used for
Glucose to G-6P - hexokinase and fructose -6P to fructose 1 -6 BP phosphofructokinase -1
Leu - lys
Poor defense from oxidizing agents - fava beans - sulfonamides - primaquine - antituberclosis drugs leadig to hemolytic anemia.
Inhibits acetaldehyde dehydrogenase and contributes to hangover symptoms

4. How do odd chain fatty acids participate in gluconeogenesis
IMP precursor
Via 1 proprionyl - CoA which can enter the TCA as succinyl - CoA and undergo gluconeogenesis
Fructose 1 -6 bisphosphate
Metachromatic leukodystrophy - arylsulfatase A - cerebroside sulfate AR

5. What does apoCII do
Pseudomonas and s aureus
NADH - NADPH - FADH2
Cofactor for LPL
Animal products - synthesized only by microorganisms - large reserve pool - mainly in liver

6. What are the findings in Down's syndrome
Inhibits RNA polymerase II - found in death cap mushrooms
Anabolic processes as a supply of reducing equivalents
Foliage - small reserve in liver - eat green leaves
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

7. What inhibits pyruvate kinase
Locus heterogeneity - ocular albinism is x- linked recessive
Transfers methyl units
ATP and alanine
Basement membrane or basal lamina

8. CF - is AR deletion In what gene On what chromosome resulting in a deletion inf what?
CFTR gene - 7 - Phe 508
Hyperlipidemia
Huntingtons
Cheilosis - inflammation of lips - scaling and fissures at the corner of the mouth - corneal vascularization

9. What does a kinase do
Fatty acid oxidation - acetyl - Coa production - TCA cycle - OXPHOS
Uses ATP to add high energy phophate group onto substrate
Degradation of TG stored in adipocytes
B48 - AIV - CII - E

10. What are the glucogenic/ketogenic amino acids
Macrocytic - megaloblastic anemia - no neurologic sx - most common vit def in the US - seen in EtOH and pregs
Y shaped region along the DNA template where leading nad lagging strands are synthesized
Ile - phe - thr - trp
Transfers cholesterol from mature HDL to VLDL - IDL and LDL (cholesterol ester transfer protein)

11. What else can phosphoylate phosphorylase kinase
Ca/calmodulin in muscle to coordinate with muscle activity
CTG
Core proteins
Dermatitis - alopecia - enteritis

12. What are Heinz bodies
Catalase positive (catalase neg produce H2O2 the cell can use) like S. aureus or aspergillus
NH2-(C=O) - NH2 one NH2 from ammonia the C=O from CO2 - and the other NH2 from aspartate
Basement membrane or basal lamina
Oxidized hemoglobin precipiated within RBCs

13. How does chloramphenicol work
IDL
Inhibits 50S peptidyltransferase
High output cardiac failure - dilated cardiomyopathy - edema
Comlex II

14. In which structures do you find microtubules
Anchor muscle fibers - primarily in skeletal and cardiac muscle
Flagella - cilia - mitotic spindles
Protective against malaria
Rotenone - CN- - antimycin A - CO

15. What is the RDE of gluconeogenesis
Neg to pos
Attachment of ubiquitin to defective proteins tag them for breakdown
Fructose 1 -6 bisphosphate
Alpha 1 -4 glucosidase

16. What is the defectin IV - hypertriglyceridemia
Antioxidant - constituent of visual pigment - essential for nl differntiation of epithelial cells into specialized tissue (pancreatic cells - mucus secreting cells) used to treat measles
Ile - phe - thr - trp
Neg to pos
Hepatic overproduction of VLDL causing pancreatitis - elvelated TGs and VLDL

17. What tissues have only aldose reductase
Lariat shape in order and remove intron precisely and join 2 exons
Schwann cells - lens - retina - kidneys
EtOH dehydrogenase and acetaldehyde dehydrogenase
Alkaptonuria - may have debiliating arthralgias

18. What apolipoprotein is on LDL
B100
Provide a source of NADPH from an abundantly available glucose 6P - create ribose for nucleotide synthesis and glycolytic intermediates
Glucose to G-6P - hexokinase and fructose -6P to fructose 1 -6 BP phosphofructokinase -1
Orotate precursor - with PRPP added later

19. What does apoB100 do
Neural tube
5' to 3'
Binds to LDL receptor - mediates VLDL secretion
P2 +2pq+ = 1

20. What is kartageners syndrome
Immotile cilia due to dynein arm defect - male and femail infertile - bronchiectasis - recurrent sinusitis - associated with situs inversus
N to C
Cystic medial necrosis of aorta leading to aortic incompetence and dissecting aortic aneurysms - floppy mitral valve - subluxation of lenses
2 rings

21. What are the products for glycolysis
Alkaptonuria - may have debiliating arthralgias
Gamma carboxylation of glutamic acid residues on various proteins concerned with blood clotting - synthesized in intestinal flora
By inhibiting formation of the initiation complex and cause misreading of mRNA
2 pyruvate - 2ATP - 2NADH - 2H+ - 2H2O

22. What is Gowers maneuver
Protein
Mental retardation - hyperphagia - obesity - hypogonadism - hypotonia - deletion of normally active paternal allele on chromosome 15
Assistance of upper extremities to stand up
Inhibit DNA gyrase specific for prokaryotic topoisomerase

23. What neuroanatomical strutures are injured in wernicke - korsakoff
5' to 3'
Marfans
Metanephrine
Medial dorsal nucleus of thalamus - mamillary bodies

24. What shape does the RNA generate during splicing and why?
Tyrosine
Inherited lysosomal storage disorder; failure of addition of mannose 6 phosphate to lysosomal proteins - enzymes secreted outside of cell instead of lysosomes
Change resulting in early stop codon
Lariat shape in order and remove intron precisely and join 2 exons

25. What does apoA 1 do
Peroxide
Activates LCAT
Enhancers
Neural tube

26. Which anticancer drugs work on microtubules
Cheilosis - inflammation of lips - scaling and fissures at the corner of the mouth - corneal vascularization
Transmitted only through mother - all offspring of affected females may show signs of disease
Vincritsine/vinblastine
Inc melt - dec fluidity

27. The pyruvate dehydorgenase complex serves In what reaction: reactants
Pyruvate - NAD+ - CoA
B6
Ribulose 5P to ribose 5P - G3P and F6P - transketolase and B1
Mediates extra remnant take up

28. How is ammonium transported from muscle to the liver for urea cycle
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
Rotenone - CN- - antimycin A - CO
Provide a source of NADPH from an abundantly available glucose 6P - create ribose for nucleotide synthesis and glycolytic intermediates
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

29. How does warfarin work
Pyruvate dehydrogenase - ATP - NADH - acetyl - CoA
Vit K antagonist
Zero
Oxidized hemoglobin precipiated within RBCs

30. What ribosomes do eukaryotes have
40 - 60 - 80
Silencers
Oxidative and nonoxidative - no ATP produced or used
Medial dorsal nucleus of thalamus - mamillary bodies

31. Type I bone
Ca/calmodulin in muscle to coordinate with muscle activity
Orotate precursor - with PRPP added later
Degredation of dietary TG in small intestine
BOne

32. What is the RDE of fatty acid oxidation
ADEK - dependent on gut (ileum) and pancreas - accumulate in fat and can cause toxicity
Carnitine acyltransferase I
OTC has hyperammonemia - orotic aciduira does not
Fed

33. What defects characterize velocardiofacial syndrome
Delayed wound healing - hypogonadism - dec in adult hair - dysguesia - anosmia - may predispose to EtOH cirrhosis
Palate - facial and cardiac defects
Binds 50S - blocking translocation
Inhibit DNA gyrase specific for prokaryotic topoisomerase

34. What converts dopamine to NE
Malate - aspartate shuttle or the glycerol 3 phosphate shuttle
Dopamine beta hydroxylase
Nonsense > missense > silent
Neimann - pick - sphingomyelinase - sphingomyelin - AR

35. What CETP do
Transfers cholesterol from mature HDL to VLDL - IDL and LDL (cholesterol ester transfer protein)
EtOH dehydrogenase and acetaldehyde dehydrogenase
Lariat shape in order and remove intron precisely and join 2 exons
Malate - aspartate shuttle or the glycerol 3 phosphate shuttle

36. What is the rate limiting enzyme in cholesterol synthesis
Glucose to G-6P - hexokinase and fructose -6P to fructose 1 -6 BP phosphofructokinase -1
Inc vit B6
Production of a recombinant DNA molecule that is self perpetuating - plasmids - selection - restriction enzyme cleavage - tissue mRNA with reverse transcriptase to make cDNA
HMG- CoA (HMG- CoA to mevalonate

37. cytoskeletal elements associated with cilia - flagella - mitotic spindle - neurones and centrioles
Microtubules
Macrocytic - megaloblastic anemia - hypersegmented PMNs - neurologic symptoms - (parasthesias - subacute combined degeneration) due to abnl myelin - prolonged becomes irreversible
Promotor - TATA box - and CAAT box - AT rich
Diphyllobothrium latum

38. What co - factors are required for the pyruvated dehydrogenase complex
HMG- CoA - brain to 2 molecules of acetyl - Coa - excreted in urine
Pyrophosphate (B1 - thiamine - TPP) FAD (B2 - riboflavin) - NAD (B3 - niacin) - CoA (B5 pantothenate) - lipoic acid
Directly inhibit the Na/K pump which leads to indirect inhibition of Na/Ca exchange - inc calcium inside the cell - inc cardiac contractility
Muscle

39. What does DNA ligase do
Seals.
Progressive hreditary nephritis and deafness - associated with occular disturbances - due to abnl type IV collagen - usually x- linked recessive
Glossitis - severe = pellagra - diarrhea - dermatitis - dementia
Phosphorylation excess glucose to sequester it - liver becomes blood glucose buffer

40. What do the single stranded binding proteins do
3' end (with CCA)
Post to neg
Prevent strands from reannealing
Degredation of TG circulating in chylomicrons and VLDLs

41. Which antifungal drugs work on microtubules
IDL
Griseofulvin
Glycolysis - fatty acid synthesis - HMP shunt - protein synthesis (RER) - steroid synthesis (SER)
Malonyl coa

42. If two alleles are present - but the active allele is deleted - what happens
Base + ribose + phosphate (3' -5') phosphodiester bond
Zero
Disease
Confusion - opthalmoplegia - ataxia - confabulation - personality changes and memory loss

43. Which carbon bears the triphosphate and the energy source for bond formation

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44. What is the activated carrier for phosphoryl
Alanine
ATP
Tryptophan excretion in urine and dec absorption from the gut leading to pellagra
Receptor tyrosine kinases - protein phosphatase - takes phosphate off glycogen phosphorylase kinase inactivating it

45. What rxn creates ATP
Inhibits lipoic acid - vomiting - rice water stools - garlic breath
Thymic - parathyroid and cardiac
Phosphenolpyruvate to pyruvate catalyzed by pyruvate kinase
Thiamine - in thiamine pyrophosphate a cofactor for pyruvate dehydrogenase (glycolysis) - alpha ketoglutarate dehydrogenase (TCA cycle) - transketolase (HMP shunt) - and branched - chain AA dehydrogenase

46. What lab value can distinguish orotic aciduria from ornithine transcarbamoylase def?
BOne
ATP - citrate
Terminal regions - tropocollagen
OTC has hyperammonemia - orotic aciduira does not

47. What does beta oxidation do and Where does it occur
Breaks down acyl - coa to acetyl coa groups in mito
Flank pain - hematuria - HTN - progressive renal failure
Dec phenylalanine (contained in aspartame - Nutrasweet) inc tyrosine in diet
Inc intestinal absorption fo calcium and phosphate - inc bone resorption

48. Of the four possible fates for pyruvate - which ends anaerobic glycolysis as in RBCs - leukocytes - kidney medulla - lens - testes and cornea
X- linked recessive
Sucrose = glucose + fructose - lactose = glucose + galactose
Lactate
Mcardle's - skeletal muscle glycogen posphorylase

49. What substance inside the cells replenishes NADPH
Facial flushing
Regulatory proteins that control cell cycle events; phase specific; activate CDKs
Pyrophosphate (B1 - thiamine - TPP) FAD (B2 - riboflavin) - NAD (B3 - niacin) - CoA (B5 pantothenate) - lipoic acid
G6PD

50. What is the amino acid precursor for histamine
In mitochondria - pyruvate to oxaloacetate - requires biotin - ATP - activated by acetyl coA
Histidine
25OHD3
1 ring