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final

Quiz yourself by thinking what should be in each of the black spaces below before clicking on it to display the answer.
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Question
Answer
Rifampicin   interferes with initiation of transcription by blocking the passage channel fo RNA/DNA hybrid, prok only  
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Actinomycin   inserts itself btwn base pairs (DNA can't be a template for RNA pol) both prok and eukaryotes  
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streptolydigin   binds to RNA pol, prevents phosphodiester bond formation (prok)  
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α-amanitin   strongly inhibits RNA polymerase II, weakly inhibits RNA pol III (euk)  
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streptomycin   inhibits initiation, misreading of mRNA (prok)  
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tetracycline   binds to 30s, inhibits binding of aminoacyl-tRNA (prok)  
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chloramphenicol   inhibits peptidle transferase at 50s (prok)  
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cyclohexamide   inhibits peptidyl transferase activity in 60s (euk)  
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Erythromycin   binds to 50s subunit and inhibits translocation  
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Puromycin   premature chain termination, acts as an analog of aminoacyl-tRNA (prok & euk)  
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Cyclin D   CDK 6&4 get through G1  
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Cyclin E   CDK 2 G1--> S  
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Cyclin A   S-->G2-->M, regulated by APC  
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Cyclin B   CDK1 through M, regulated by APC  
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Retinoblastoma   Rb is a tumor suppressor gene, usually binds & inactivates E2F, with growth signals Rb is phosphorylated and releases E2F (transcription factor)  
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p53   transcription factor, ordinarily synthesized and degraded, if it hangs out to long it moves into the nucleus and turns on transcription of p21 (CDK-inhibitor)  
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Li_Fraumeni Syndrome   inherited p53 defect  
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autosomal dominant   1. no skipping of generations 2. males and females are equally likely to be affected 3. normal siblings of affected individuals do NOT pass it on 4. usually a structural protein not an enzyme  
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Marfan Syndrome   Autosomal dominant (variable expressivity), mutation in the FBN1 gene, chrom 15 - encodes for fibrillin-1, skeletal abnormalities  
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Huntington   Autosomal dominance (late onset), CAG repeats in the coding section (encoded bc of strand slippage during replication)  
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myotonic dystrophy   autosomal dominant, delayed expression in NON-translated region, slowly progressive muscle weakness and myotonia  
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genetic anticipation   tendency for the severity of the disease to increase and the age of onset to decrease  
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fragile x   X-linked dominant increased CGG repeats, FMR1 not expressed, no protein made, since females carry 2 Xs tends to be milder  
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neurofibromatosis type 1   cafe au lait spots, protein is neurofibromin which causes a loss of growth supressors, autosomal dominant inheritance (high new mutation rate)  
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achondroplasia   autosomal dominant, high recurrent mutation, short stature, mutation in the fibroblast growth factor receptor gene 3 (severely shortened bones)  
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Potter's syndrome   autosomal dominant, incomplete penetrance  
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Autosomal recessive   1. equal male & female 2. sibling recurrence rate is 1/4 3. characteristically found in siblings, not parents 4. parents may be related 5. may be an isolated event in small sibships  
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X-linked recessive   never pass father to son affected females MUST have affected fathers typically passed from affected grandfather to 1/2 of grandsons  
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X-linked dominan   never pass father to son all daughters of affected parents are affected, all sons of affected male and unaffected female are normal affected females x normal male = 1/2 affected sons male more seriously affected than females  
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Patau   trisomy 13, usually die within 3 months  
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edwards syndrome   trisomy 18, distinct facial features, 80% of those affected are females,  
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klinefelter   XXY low levels of testosterone  
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turner   XO, underdeveloped female secondary sex characteristics, webbed neck  
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williams syndrome   deletion of the q arm of chrom 7 (including elastin gene), broad forehead, short nose, widely spaced teeth  
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cri du chat   deletion of the short arm of chrom 5, 80% of time comes from father's sperm,abnormal larynx development = mewing (becomes normal w/i few weeks),  
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imprinting   reversible form of inactivation, acts through methylation, only occurs on 9 chrom  
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prader-willi   small deletion on the short arm of chrom 15 from father. hypotonia, difficulty feeding, insatiable appetite, small hands and feet, hypogonadism  
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angelman   small deletion on short arm of chrom 15 from mother, severe mental retardation, nonverbal, balence disorder  
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osteogenesis imperfecta   mosaicism, type 1 collagen disorder, easy fractures of the bone, autosomal dominant  
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methotrexate and aminopterin   inhibit thymidylate synthase (no dTTP)  
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fluorouracil   inhibits dihydrofolate reductase (do dTTP)  
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HGPRT   deficiency --> Lesch Nyan, X-linked recessive  
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Adenosine deaminase deficiency   used in purine salvage pathway, causes SCID, ATP and dATP accumulate --> imbalnce of nucleotide pool, prevents DNA syn  
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glutamine PRPP amidotransferase   purine synthesis, removes pyrophosphate from PRPP leavind a phosphoribosyl amine  
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Xanthylate synthase   uses NAD and H2O to form a carbonyk, can be inhibited by GMP  
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allopurinal   suicide inhibitor of xanthine OXIDASE, enzyme can't degrade purines to urate  
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carbamoyl phosphate synthase   pyrimidine synthesis, NOT from urea cycle, inhibited by UTP, activated by PRPP  
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ribonucleotide reductase   removes 2'OH, so they can be incorporate into DNA, regulated to keep the dNTPs in balence  
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campothecin and doxorubicin   type 1&2 topoisomerase inhibitors, stabilize the DNA-topoisomerase complex, DNA makes cut and the drug blocks the reattachment  
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cohesins   hold chromosomes together, regulated by APC (through securin and separase)  
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T-loops   hides dsDNA from DNA repair machinery, free of nucleosomes, stabilized by TRF1 proteins  
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RNA syn   does NOT require a promoter, initiation is TATA region, termination is GC rich (forms hairpin), can also have rho factor (ATPase and a helicase, binds to Crich, G poor region and pulls it away from the RNA pol and DNA template)  
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RNA trasncription   a DAB of F brings RNAPOL, EH?  
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spliceosom   snRNPs and pre-mRNA  
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B-thalassemia   abnormal splicing of B-globin  
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amino-acyl tRNA stnthetase   requires ATP  
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ran   signal peptide receptor in nucleus and cytoplasm, serves in both nuclear import and export,  
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NF-AT   transcription factor mediated by nuclear import/export, wehn it is stimulated calcinurin moves to the nucleus and stimulates trascription, in low calcium environment calcineurin falls off and the NF-AT is kicked out of the cell  
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cholera   colonize the small intestine and produce mono-ADP-ribotransferase, modifies the G-protein involved in walter and salt balence. G-protein cannot release GTP so Adenylyl cyclase is active --> inc cAMP --> intestinal cells secrete water and salts  
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Pertussis   whooping cough, colonize the lungs and add ADP-ribose ot G-protein resulting in inc mucus secretion  
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Diphtheria   infect nasopharynx/skin, toxin is endocytosed after binding to epidermal growth factor receptor --> acidified in endosome --> ribosylation of EF2 (blocks protein syn)  
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Ricin   heterodimeric ribosome-inhibiting proteinm doens't use NAD+ instead cleaves A and stops protein syn  
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somatic cell hybrids   use sendai virus to fuse 2 cells and nuclei from different species. can't be used for disease genes  
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RFLP   markers to certain locations on different chromosomes, can indicate whether crossing-over has occurred btwn the marker and the disease gene (show you how close together the 2 are)  
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Beckswith Wiedemann Syndrome   chrom 11 - imprinted in father, maternal deletion = syndrome, paternal deletion = no effect  
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CGH comparative genomic hybridization   detects global gains and losses by compaing sa,ple DNA to the normal human chrom, can detect anueploidy or gene amplificaion in tumors, will not detect balenced rearrangements  
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GPCRs   largest family of cell surface receptors, 7transmembrane domains, extracellular domain binds ligand, can be inactivated by receptor inactivation, receptor internalization or receptor downregulation  
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ATP used per peptide bond in protein synthesis   4  
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Neurofibromatosis II   mutation on tumor supressor gene, chrom 22, Bilateral acoustic neuroma (CN VIII), Juvenile cateracts  
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Rett's   MECP2 genes>> form synapses between nerve cells, unclear, X-linked, fatal in males, hand-wringing  
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Beckwith- Wiedemann   Macrosomia: large body Macroglossi: large tongue Enlargement of internal organs maternally imprinted Wilm’s tumor and embryonal tumors omphalocele  
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Xeroderma pigmentosa   inability to repair thymine dimers  
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Ataxia telangectasia   defect in ATM protein>> protein kinase activated by double strand breaks  
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Wilm’s tumor   defect in the imprinting of the insulin like growth factor 2 gene>> both maternal and paternal expression Symptoms: Childhood tumor of the kidney  
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karyotyping with light microscopy   G-banding  
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