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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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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 converts limit dextran to glucose
Infection - free radicals generated by inflammatory response
Antigen - antibody reactivity - can look for antigen or antibody in patients blood
Debranching enzyme
Cytosol

2. Where is glucokinase found - What are the Km and Vmax - and what induces it
L form
Antioxidant - constituent of visual pigment - essential for nl differntiation of epithelial cells into specialized tissue (pancreatic cells - mucus secreting cells) used to treat measles
Terminal regions - tropocollagen
Liver and beta cells of pancreas - low affinity - high Km - high capacity - high Vmax - induced by insulin

3. What does Alports syndrome cause and why
Citrate - isocitrate - alpha ketoglutarate - succinyl - CoA - succinate - fumarate - malate - oxaleoacetate
Oxidative is irreversible
Alkaptonuria - may have debiliating arthralgias
Progressive hreditary nephritis and deafness - associated with occular disturbances - due to abnl type IV collagen - usually x- linked recessive

4. What is the TX for pyruvate dehydrogenase deficiency
Medial dorsal nucleus of thalamus - mamillary bodies
AMP - fructose 2 -6 BP
Inc intake of ketogenic nutrients - high in fact content or inc lysine or leucine
Glycogen synthase

5. What happens with dry beriberi
Polyneuritis - symmetrical muscle wasting
Galactitol - galactose appears in blood and urine - can cause infantile cataracta - AR
NADPH
Nonreciprocal chromosomal translocation that commonly involves chromosome pairs 13 - 14 - 15 - 21 - 22

6. Of the four possible fates for pyruvate - which one carries amino groups to liver from muscle
Prokaryotic only - degrades RNA primer and fills in the gap with DNA
Griseofulvin
Alanine
Catalase positive (catalase neg produce H2O2 the cell can use) like S. aureus or aspergillus

7. What lab procedure is used to amplify desired fragment of DNA and What are the 3 steps
PCR - denaturation - annealing - elongation
G6PD
By inhibiting formation of the initiation complex and cause misreading of mRNA
Thymic - parathyroid and cardiac

8. What are the glucogenic essential amino acids
Met - val - arg his
PCR - denaturation - annealing - elongation
Huntingtons
Alpha - ketoglutarate dehydrogenase complex

9. What is a frame shift
2 pyruvate - 2ATP - 2NADH - 2H+ - 2H2O
Wernicke - korsakoff - dry and wet beriberi
Change resulting in misreading of all nucleotides downstream - usually resulting in a truncated nonfxnal protein
Trisomy 13 - severe mental retardation - rocker bottom feet - micophthlamia - microcephaly - cleft lip/palate - holoprosencephaly - polydactyly - congenital heart disease - death usually within the first year

10. What is the RDE of gluconeogenesis
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
Krabbes - galactocerebrosidase - galactocerebroside - AR
Fructose 1 -6 bisphosphate
O- oligosaccharaides

11. Type II cartilage
ATP and methionine
CarTWOlage
Convulsions - hyperirritability - peripheral neuropathy - deficiency inducible by INH and oral contraceptives - sideroblastic anemais
Neimann - pick - sphingomyelinase - sphingomyelin - AR

12. Giving folate during early pregnancy is important to prevent what birth defects
Failure to track objects or develop a social smile
More than 1 codon may code for the same amino acid
75 to 90 - cloverleaf
Neural tube

13. What is the function of biotin
Phenylalanine hydroxylase - tetrahydrobiopterin cofactor
Cofactor of carboxyltion enzymes: pyruvate carboxylase - acetate - CoA carboxylase - and propionyl - CoA carboxylase
Read from a fixed starting point as a continuous sequence of bases
Protein kinase A

14. What apolipoproteins are on IDL
Facial flushing
Skin
Phenylalanine hydroxylase - tetrahydrobiopterin cofactor
B100 and E

15. How many rings do purines have
Gaucher's - beta glucocerebrosidase - glucocerebrosie - AR
High output cardiac failure - dilated cardiomyopathy - edema
Familial hypercholesterolemia - hyperlipidemia type IIA
2 rings

16. Describe robertsonian translocation
Liver and leafy veggies
GTP hydrolysis - initiation factors assemble 40S ribosomal subunit with initiator tRNA and are released with mRNA and ribosomal subunit assemble with the complex
Nonreciprocal chromosomal translocation that commonly involves chromosome pairs 13 - 14 - 15 - 21 - 22
Metanephrine

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

18. What enzyme converts phenylalanine to tyrosin
Athralgias - fatigue - headaches - skin changes - sore throat - alopecia
Mebendazole/thiabendazole
Phenylalanine hydroxylase
Reads usual codon but inserts wrong AA

19. What does the CFTR channel do in the lungs - GI tract and skin
Tall with long extremeties - pectus excavatum - hyperextensive joints - arachnodactyly
Mebendazole/thiabendazole
Tryosine hydroxylase
Active secretion in lungs and GI - reabsorbs in skin

20. Which aspect of the spliceosome do patients with lupus make antibodies against
Citrate - acetyl coa from mito to cyto
SnRNPs
Pyrophosphate (B1 - thiamine - TPP) FAD (B2 - riboflavin) - NAD (B3 - niacin) - CoA (B5 pantothenate) - lipoic acid
Phenylethamolamine N methyl transferase

21. Type II collagen
X- linked recessive
Adenosine to inosine
Ubiquitous - low Km - high affinity - low capacity low Vmax - uninduced by insulin
Cartilage - hyaline - vitreous body - nucleus pulposus

22. What substances are uncouling agents
2 -4 DNP - aspirin
Oxidative and nonoxidative - no ATP produced or used
Inhibit DNA gyrase specific for prokaryotic topoisomerase
Poly A polymerase - signal is AAUAA

23. How is orotic aciduria inherited
Alkaptonuria - may have debiliating arthralgias
AR
Targets the proteins for lysosome
Regulatory proteins that control cell cycle events; phase specific; activate CDKs

24. developmental delay - gargoylism - airway obstruction - corneal clouding - hepatosplenomegaly
Glycolysis - fatty acid synthesis - HMP shunt - protein synthesis (RER) - steroid synthesis (SER)
Arginine
Vit K antagonist
Hurlers syndrome - alpha L iduronidase - heparan sulfate/dermatan sulfate - AR

25. cell signaling defect of fibroblast growth factor (FGF) receptor 3 - results in dwarfism - short limbs - head/trunk nl size - associated with advanced paternal age
NAD+
Achondroplasia
Meiotic nondisjunction of homologous chromosomes (associated with advanced maternal age) leading to trisomy 21
HMG- CoA synthase

26. What causes biotin def
Orotate precursor - with PRPP added later
Aldolase B - AR - fructose 1P accumulates causing a dec in availabel phosphate which inhibts glycogenolysis and gluconeogenesis leading to hypoglycemia - cirrhosis - jaundice and vomiting
Antibiotic use or excessive ingestion of raw eggs
Pyroxidine - converted to pyroxidine phosphate a cofactor used in transamination - decarboxylation - glycogen phosphorylase - cystathionine synthesis and heme synthesis - required for synthesis of niacin and tryptophan

27. cytoskeletal elements associated with vimenentin - desmin - cytokeratin - glial fibrillary acid protiens (GFAP) - neurofilaments
IDL
Intermediate filaments
Inhibits 50S peptidyltransferase
Tuberous sclerosis

28. What does the deletion of the dystrophin gene lead to...
African Americans and Asians
By inhibiting formation of the initiation complex and cause misreading of mRNA
Accelearted muscle breakdown
Von Gierke's - Pompe - Cori - McArdle

29. What are the products for glycolysis
Delayed wound healing - hypogonadism - dec in adult hair - dysguesia - anosmia - may predispose to EtOH cirrhosis
Polyneuritis - symmetrical muscle wasting
2 pyruvate - 2ATP - 2NADH - 2H+ - 2H2O
Attachment of ubiquitin to defective proteins tag them for breakdown

30. What co - factors are required for the pyruvated dehydrogenase complex
HMG- CoA - brain to 2 molecules of acetyl - Coa - excreted in urine
Phosphofructokinase 1
Mental retardation - growth retardation - seizures - fair skin - eczema - musty body odor
Pyrophosphate (B1 - thiamine - TPP) FAD (B2 - riboflavin) - NAD (B3 - niacin) - CoA (B5 pantothenate) - lipoic acid

31. What are the 2 distinct phases of the HMP shunt and How many ATP are used and produced
Nonsense > missense > silent
Oxidative and nonoxidative - no ATP produced or used
P+q = 1
Superoxide dismutase

32. What apolipoproteins are on chylomicrons
AMP - fructose 2 -6 BP
Degredation of dietary TG in small intestine
ATP
B48 - AIV - CII - E

33. What inhibits the carnitine shuttle
Lacks glucose 6 phophatase
Mcardle's - skeletal muscle glycogen posphorylase
Malonyl coa
Ca/calmodulin in muscle to coordinate with muscle activity

34. Name as many x- linked recessive disorders as you can

35. How does insulin inhibit glycogenolysis
Oral uridine administration
Receptor tyrosine kinases - protein phosphatase - takes phosphate off glycogen phosphorylase kinase inactivating it
Thiamine - in thiamine pyrophosphate a cofactor for pyruvate dehydrogenase (glycolysis) - alpha ketoglutarate dehydrogenase (TCA cycle) - transketolase (HMP shunt) - and branched - chain AA dehydrogenase
In mitochondria - pyruvate to oxaloacetate - requires biotin - ATP - activated by acetyl coA

36. What does GFAP stain for
Neuralgia
Oligomycin
OTC has hyperammonemia - orotic aciduira does not
Confusion - opthalmoplegia - ataxia - confabulation - personality changes and memory loss

37. 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
DsRNA promotes degradation of target mRNA knocking down gene expression
Williams syndrome
Inc insulin - dec cAMP - dec PKA
Failure to track objects or develop a social smile

38. What chromosome is the NF gene on...
Medial dorsal nucleus of thalamus - mamillary bodies
17 - 17 letters in von Recklinghausen
FAP
Seals.

39. What is the breakdown product of epi
Prevent strands from reannealing
III - joint dislocation - anuerysms - organ rupture
Blood - bone marrown - amniotic fluid - placental tissue
Metanephrine

40. RNA poly can't proofread - but What can it do
Same as sprint + OXPHOS
Type II
Intermediate filaments
Initiate chains

41. What clotting factors require vit K
Pyruvate dehydrogenase - ATP - NADH - acetyl - CoA
II - VII - IX - X (1972) protein C and S
Keep glutathione reduced so it can detoxify free radicals and peroxides
NAD+

42. In mismatch repair - which strand is recognized and In what hereditary syndrome is this defective
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
Marasmus - muscle wasting
Unmethylated - newly synthesized - HNPCC
Coenzyme A - lipoamide

43. What two proteins make up microtubules and how are they arranged
Males are infertile due to bilateral absence of vas deferens
Sulfation
Alpha and beta tubulin - dimers have two GTP bound
Inhibits acetaldehyde dehydrogenase and contributes to hangover symptoms

44. What kind of branches do glycogen branches have
Scrutinizes - hydrolyzes the bond
Glucose 6 phosphate dehydrogenase (G6PD)
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
Alpha 1 -6 and alpha 1 -4

45. What are the blood glucose levels maintained by for days 1-3
Pyroxidine - converted to pyroxidine phosphate a cofactor used in transamination - decarboxylation - glycogen phosphorylase - cystathionine synthesis and heme synthesis - required for synthesis of niacin and tryptophan
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)
Liver - also in kidney and gut epithelium
Inhibits RNA polymerase II - found in death cap mushrooms

46. 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
FISH
African Americans and Asians
Wrinkles and acne
By inhibiting formation of the initiation complex and cause misreading of mRNA

47. What does beta oxidation do and Where does it occur
Neimann - pick - sphingomyelinase - sphingomyelin - AR
Adenylyl cycle - inc cAMP - inc PKA - glycogen phosphorylase kinase activated - glycogen phosphoylase active - glycolysis
Adenosine to inosine
Breaks down acyl - coa to acetyl coa groups in mito

48. What are the two possible causes of albinism
Isocitrate dehydrogenase
HDL
Lack of tyrosinase (no melanin) AR ordefective tyrosine transporters (less tyrosine) - can results in lack of migration of NC cells
Glycogen and FFA oxidation; glucose conserved for final sprinting

49. What feedback inhibits hexokinase
Y shaped region along the DNA template where leading nad lagging strands are synthesized
Glucose -6 phosphate
LCAT (lecithin cholesterol acyltransferase)
Tryptophan

50. Who typically has lactase def
Microtubules
Failure to track objects or develop a social smile
Cofactor of carboxyltion enzymes: pyruvate carboxylase - acetate - CoA carboxylase - and propionyl - CoA carboxylase
African Americans and Asians