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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. What is the RDE of glycogenolysis
Oligomycin
Liver and beta cells of pancreas - low affinity - high Km - high capacity - high Vmax - induced by insulin
B6
Glycogen phosphorylase

2. What is the activated carrier for methyl groups
ATP
SAM
Chylomicrons
Accelearted muscle breakdown

3. What bone disorder has x linked dominant inheritance
Hypophophatemia rickets - vit D resistant ricketts - inc wasting of phosphate in proximal tubule - rickets like presentation
4 under the floor
Hepatic glycogenolysis - adipose tissue release of FFA - muslce and liver FFA - hepatic gluconeogenesis from peripheral tissue lactate and alanine and from adipose tissue glycerol and proprionyl - coA (odd chain FFA)
Alcohol version of glucose - can trap glucose in cell - aldose reductase

4. The pyruvate dehydrogenase complex serves In what reaction: products
Acetly- CoA - CO2 - NADH
Tryptophan excretion in urine and dec absorption from the gut leading to pellagra
Miscarriage - stillbirth - chromosomal imbalance (down - patau)
Inc dicarboxylic acids - dec in glucose and ketones

5. What happens to oxaloacetate in alcholism
Oxidizes substrate
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
Excess NADH shunts oxaloacetate to malate (backwards) stalling the TCA and shunting glucose and FFA to ketone body production
Neonatal hemorrhage with inc PT and PTT - but nl bleeding time - can also occur after prolonged use of broad spectrum Abx

6. The pyruvate dehydorgenase complex serves In what reaction: reactants
NADPH
Chylomicrons
Pyruvate - NAD+ - CoA
Neuralgia

7. What co - factors are required for the pyruvated dehydrogenase complex
Exons
Galactitol - galactose appears in blood and urine - can cause infantile cataracta - AR
Myeloperoxidase
Pyrophosphate (B1 - thiamine - TPP) FAD (B2 - riboflavin) - NAD (B3 - niacin) - CoA (B5 pantothenate) - lipoic acid

8. What is the pathway in the fed state leading to dec FBPase -2 and inc PFK-2
TRNA
Tay- Sachs - hexosaminidase A - GM2 ganglioside - AR
Arg and his inc in histones Which bind negatively charged DNA
Inc insulin - dec cAMP - dec PKA

9. What happens do glycogen in skeletal muscle during exercise
Glycogenolysis to form glucose
CGG
Antioxidant - constituent of visual pigment - essential for nl differntiation of epithelial cells into specialized tissue (pancreatic cells - mucus secreting cells) used to treat measles
Palate - facial and cardiac defects

10. A small proportion of Down syndrome is due to What two genetic events
HMG- CoA synthase
Robertsonian translocation and mosaicism
Liver hepatocytes and steroid producing cells of the adrenal cortex
Colchicine

11. What activates the pyruvate dehydrogenase complex
5' to 3'
In cytosol - oxaloacetate to phosphenolpyruvate - requires GTP
Tendency for certain alleles at 2 linked loci to occur together more often than expected by chance - varies is different populations
Exercise: inc NAD/NADH - inc ADP - inc Ca

12. What is the fxn of vit E
Antioxidant - protects RBCs and membrances from free radical damage
BOne
Galactitol - galactose appears in blood and urine - can cause infantile cataracta - AR
Removes phosphate group from substrate

13. What apolipoproteins are on IDL
Von hippel lindau - 3
B100 and E
Galactitol - galactose appears in blood and urine - can cause infantile cataracta - AR
Reticulin - skin - blood vessels - uterus - fetal tissue - granulation tissue

14. What reaction does adenosine deaminase normally catalyze
Fructose via sorbitol dehydrogenase - inc sorbitol leading to osmotic damage as in cataracts - retinopathy - peripheral neuropathy as in diabetes
Pyruvate - NAD+ - CoA
Adenosine to inosine
Antigen - antibody reactivity - can look for antigen or antibody in patients blood

15. How is ATP used by the cell
Tendency for certain alleles at 2 linked loci to occur together more often than expected by chance - varies is different populations
ATP hyrdolysis couple to energetically unfavorable rxns
3 NADH - 1 FADH2 - 2 CO2 - 1 GTP = 12 ATP (x2 per glucose)
Never go to G0 - divide rapidly with a short G1 - bone marrow - gut epi - skin - hair follicles

16. Milder form of type I with nl blood lactate levels - dz and enzyme

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17. Outside the fibroblast What part of procollagen is cleaved and What is it transformed into
Terminal regions - tropocollagen
Asp and Glu
Hyperlipidemia
NAD+

18. How do cardia glycosides work
NF2 on chromosome 22
Intermediate filaments
Acetly- CoA - CO2 - NADH
Directly inhibit the Na/K pump which leads to indirect inhibition of Na/Ca exchange - inc calcium inside the cell - inc cardiac contractility

19. Eukaryotic and prokaryotic DNA synthesis is blank and involves both blank and blank strands
Inability to transport LCFAs into mitochondria results in toxic accumulation causing weakness - hypotonia - hyperketotic hyperglycemia
Microtubules
Semiconservative - continuous and discontinuous strands (okazaki fragments)
25OHD3

20. What substances inhibit phosphofructokinase -1
Receptor tyrosine kinases - protein phosphatase - takes phosphate off glycogen phosphorylase kinase inactivating it
ATP - citrate
Macrocytic - megaloblastic anemia - no neurologic sx - most common vit def in the US - seen in EtOH and pregs
Not all individuals with a mutant genotype show the mutant phenotype

21. Delivers hepatic cholesterol to peripheral tissues - formed by LPL modification of VLDL in the peripheral tissue - taken up by target cells via RME
Prevent strands from reannealing
Griseofulvin
Mental retardation - seizures - ataxia - inappropriate laughter - deletion of normally active maternal allele
LDL

22. What does the primary transcript combine with to form the spliceosome
Inhibits acetaldehyde dehydrogenase and contributes to hangover symptoms
Malapsorption syndromes like sprue or CF or mineral oil intake
SnRNPs and other proteins
Blood - bone marrown - amniotic fluid - placental tissue

23. Where is glucokinase found - What are the Km and Vmax - and what induces it
Liver and beta cells of pancreas - low affinity - high Km - high capacity - high Vmax - induced by insulin
Malonyl coa
Malate - aspartate shuttle or the glycerol 3 phosphate shuttle
Lysine and leucine

24. What does commaless - nonoverlapping genetic code refer to...
25OHD3
Seals.
Read from a fixed starting point as a continuous sequence of bases
Nucleotide excision repair - xeroderma pigmentosum; unable to repair thymidine dimers caused by UV light

25. What does the start codon code for in eukaryotes and prokaryotes
VLDL
Degradation of TG stored in adipocytes
Eu - methionine - pro - formyl - methionine
Hypoglycemia

26. What does GFAP stain for
Liver and leafy veggies
Neuralgia
Propionyl - CoA to methylmalonyl - CoA (3C to 4C)
Y shaped region along the DNA template where leading nad lagging strands are synthesized

27. What causes Hartnup's disease
Degradation of TG remaining in IDL
UDP glucose pyrophosphorylase
1 kind with multiple subunits
AR disorder characterized by defective neutral amino acid transporte on renal and intestinal epith cells

28. What does a defective Cl channel do
Glucose 6P to CO2 - 2NADPH - ribulose 5P - G6PD - rate limiting step
Inc permeability of membrane causing a dec in proton gradient and inc in O2 consumption - ATP synthesis stops - but electron transport continues to produce heat
Secretion of abnl thick mucus that plugs lungs - pancreas - liver
NH2-(C=O) - NH2 one NH2 from ammonia the C=O from CO2 - and the other NH2 from aspartate

29. What can excess vit B3 cause
Facial flushing
ATPase that links peripheral 9 doublets and causes bending of cilium by differential sliding of doublets
Hypoglycemia
Fabrys - alpha galactosidase A - ceramide trihexoside - XR

30. What does a kinase do
Arginine
SnRNPs and other proteins
Glucose -6 phosphate
Uses ATP to add high energy phophate group onto substrate

31. Which part of the DNA binds RNA polymerase and multiple other TFs upstream from gene locus
Glucose - 2Pi - 2ADP - 2NAD+
Each codon specifies only 1 amino acid
Promotor - TATA box - and CAAT box - AT rich
Elastase - inhibited by alpha1 antitrypsin

32. What is imprinting and give an example

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33. cytoskeletal elements associated with cilia - flagella - mitotic spindle - neurones and centrioles
Ile - phe - thr - trp
NADH - NADPH - FADH2
Microtubules
2 rings

34. What polar group does guanine have - and what non polar group does thymine have
Rb and p53
Reticulin - skin - blood vessels - uterus - fetal tissue - granulation tissue
Fructose via sorbitol dehydrogenase - inc sorbitol leading to osmotic damage as in cataracts - retinopathy - peripheral neuropathy as in diabetes
Ketone - methyl

35. What is the rate determining enzyme (RDE) of glycolysis
Phosphofructokinase 1
Tryptophan excretion in urine and dec absorption from the gut leading to pellagra
Robertsonian translocation and mosaicism
AMP - fructose 2 -6 BP

36. 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
B-100 - CII and E
SnRNPs
Tryptophan

37. What other complex is similar to the pyruvate dehydrogenase complex in that it has the same co - factors and generates succinyl - CoA
Lack of proper dietary therapy during pregnancy leading to microcephaly - mental retardation growth retardation - congenital heart defects
HMG- CoA reductase
Alpha - ketoglutarate dehydrogenase complex
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

38. What happens with wet beriberi
Peroxide
ATP - citrate
High output cardiac failure - dilated cardiomyopathy - edema
Targets the proteins for lysosome

39. How does ethanol induce hypoglycemia
Thiamine - in thiamine pyrophosphate a cofactor for pyruvate dehydrogenase (glycolysis) - alpha ketoglutarate dehydrogenase (TCA cycle) - transketolase (HMP shunt) - and branched - chain AA dehydrogenase
Adenylyl cycle - inc cAMP - inc PKA - glycogen phosphorylase kinase activated - glycogen phosphoylase active - glycolysis
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
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

40. Which end of the tRNA is the amino acid bound to...

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41. What ribosomes do prokaryotes have
Hartnup disease - dec tryptophan absoprtion - malignant carcinoid syndrome (inc tryptopha metabolism) and INH (dec vit B6)
Active secretion in lungs and GI - reabsorbs in skin
30 - 50 - 70
Alcohol version of glucose - can trap glucose in cell - aldose reductase

42. What part of the pre mRNA contains the actual genetic information coding for protein
Exons
Night blindness - dry skin
Q -
Microtuble polymerization defect causing a dec in phagocytosis results in recurrent pyogenic infections - partial albinism - and peripheral neuropathy

43. What enzyme esterifies 2/3 of plasma cholesterol
Thymic - parathyroid and cardiac
Glycolysis - fatty acid synthesis - HMP shunt - protein synthesis (RER) - steroid synthesis (SER)
LCAT (lecithin cholesterol acyltransferase)
Excess NADH shunts oxaloacetate to malate (backwards) stalling the TCA and shunting glucose and FFA to ketone body production

44. What happens in folate def
Ubiquitous - low Km - high affinity - low capacity low Vmax - uninduced by insulin
Secretion of abnl thick mucus that plugs lungs - pancreas - liver
Macrocytic - megaloblastic anemia - no neurologic sx - most common vit def in the US - seen in EtOH and pregs
Carnitine shuttle - acyl - coa from cyto to mito

45. What does DNA poly III do?

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46. What else can phosphoylate phosphorylase kinase
SnRNPs
Ca/calmodulin in muscle to coordinate with muscle activity
EtOH dehydrogenase and acetaldehyde dehydrogenase
Hereditary hemorrhagic telangiectasia (Osler - Weber - Rendu syndrome)

47. What does the golgi assemble proteoglycans from
Core proteins
Hurlers syndrome - alpha L iduronidase - heparan sulfate/dermatan sulfate - AR
Polyneuritis - symmetrical muscle wasting
Inhibits RNA polymerase II - found in death cap mushrooms

48. progressive neurodegeneration - hepatosplenomegaly - cherry red spot on macula - foam cells
EtOH dehydrogenase and acetaldehyde dehydrogenase
Dec phenylalanine (contained in aspartame - Nutrasweet) inc tyrosine in diet
Mucus secreting globlet cells and antibody secreting plasma cells
Neimann - pick - sphingomyelinase - sphingomyelin - AR

49. What collagen type is most frequently affected in ehlers danlos and What are common complications
Modifies N- oligosaccharides
III - joint dislocation - anuerysms - organ rupture
Von gierkes - glucose 6 phosphatase
Supply sufficient glucose to brain and RBCs and to preserve protein

50. What does osteogenesis imperfecta causes and why
Receptor tyrosine kinases - protein phosphatase - takes phosphate off glycogen phosphorylase kinase inactivating it
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
Von gierkes - glucose 6 phosphatase
Microarrays