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53 Cards in this Set
- Front
- Back
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Where are proteins made that are used within the cell?
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Free ribosomes
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Where are proteins made that need to be transported?
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Rough ER
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What is the transport signal to send proteins to:
1. Mitochondria 2. Lysosome 3. Rough ER 4. Golgi |
1. Short N-terminal sequence (HSP 90)
2. Mannose-6-phosphate 3. Pre-Pro 4. Pro (HSP70) |
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What protein patrols the body and tells cells when to undergo apoptosis?
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D-cyclins
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What is the signal for apoptosis that cells display?
What enzymes carry out apoptosis? |
phosphatidyl serine
caspases |
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90% of necrosis is due to...
Purulent necrosis is due to... Granulomatous necrosis is due to... Fibrinous necrosis is due to... |
...ISCHEMIA
...bacteria ...non-bacteria ...vasculitis, uremia, |
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caseuos necrosis is due to...
Fat necrosis is due to... The only two places fat necrosis occurs are... |
...TB
...blunt force trauma or pancreatitis ...breast and pancreas |
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Liquefactive necrosis is due to...
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abscess formation - anaerobes
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The most common cause of monosomies is...
Monosomies occur most often in... Monosomies are most often transmitted from... |
...non-disjunction
...occur most often in males ...transmitted more often from females |
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What is the chance of getting ANYTHING by chance alone?
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1-3%
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The chance of getting anything with one risk factor is...
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10%
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What are the clues for Turner syndrome?
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X0
NOT mentally retarded Webbed Neck Cystic hygroma Coarctation of aorta Shield-shaped chest Gonadal Streak |
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What clues indicate the location of a coarctation?
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If both upper extremity pulses are strong and lower extremity pulses are weak --> stenosis is after L subclavian
If differential pulses between upper extremities --> stenosis before L subclavian |
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What are the four P's associated with Patau's syndrome?
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Trisomy 13
Polydactyly Palate is high-arched Peeing system abnormality Protruding belly (Oomphalocele) |
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What are the clues for Edward's syndrome?
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Trisomy 18
Rocker bottom feet Triphalangeal thumb |
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What are the clues for Down's Syndrome?
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Trisomy 21
Mental retardation Early onset alzheimer's Higher frequency of AML - ALL still most common 20-40% have congenital heart defects |
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Describe the mechanism by which Down's patients get early alzheimer's
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Betalipoprotein E4 gets oxidized --> neurofibrillary tangles
Inhibit oxidation with Vit. E. If not enough, treat with Donepazil (AChesterase inhibitor) or Momentin (stim NMDA pathway) |
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What are the congenital heart defects that Down's patients get?
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Endocardial cushion defects - it doesn't form
VSD and ASD - failure of growth to meet in the middle VSD - failure of ventricular septum to fully extend upward ASD - failure of septum primum to fully extend downward |
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What congenital heart disease presents with cyanosis at birth? at 1 month?
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At birth: Transposition of the great arteries
1 month: Tetralogy of Fallot |
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What defects are present in Tetralogy of Fallot?
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1. Overriding aortic arch
2. pulmonary stenosis 3. Right ventricular hypertrophy 4. VSD |
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What are Tet spells?
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1. Baby - turns blue when crying b/c inc pulm pressure drives blood through VSD
2. Toddler-squats to increase PVR leading to blood shunting from L to R side through PDA and child gets a burst of O2 |
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What is the MCC of death for a patient with cancer or patient taking chemo?
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Infection
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Which drugs should I use for rapidly growing cancers? Slow-growing cancers?
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Rapid - antimetabolites
Slow - alkylating agents |
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Which drugs are nucleoside analogs? What class of chemo drugs are they?
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Ara-C
Ara -A 5FU Antimetabolites |
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Which drug is recognized by xanthine oxidase? What is its side effect?
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6-mercaptopurine
SE: gout |
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What is the mechanism of action of methotrexate?
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inhibits dihydrofolate reductase
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What other drugs inhibit dihydrofolate reductase?
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Trimethoprim and pyrimethamine
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What is another indication for methotrexate's use? What other drugs are used for this purpose?
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steroid resistant disease
Azathioprine and cyclosporine |
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What is one of the side effects of methotrexate use? What drug can be given to rescue?
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Folate deficiency --> megaloblastic anemia
Leucovorin |
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How do alkylating agents function? What is their main side effect? How is the SE treated?
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Bind double-stranded DNA
Nausea Ondansetron - serotonin blocker |
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Which alkylating agents cause Pulmonary fibrosis?
What other drugs cause pulmonary fibrosis? |
Bleomycin, Busulfan
Amiodarone, Tocainide, methotrexate, cormustine |
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How does Adriamycin work?
What is its main SE? What drug is used to rescue it? |
Releases free radicals
SE: releases free radicals in heart causing cardiac fibrosis Rescue: Desroxzasane |
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What is the main side effect of cisplatin?
What drug is used to rescue from SE? |
SE: Renal failure
Rescue: amiphostene |
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What is the main side effect of Cyclophosphamide and Isophosphamide?
What drug is used to rescue from SE? |
SE: hemorrhagic cystitis
Rescue: Mesna |
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How does hydroxyurea work?
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Inhibits ribonucleotide reductase resulting in wipeout of the bone marrow and renewal with HgF therefore used in sickle cell
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What alkylating agent is used to treat Multiple Myeloma?
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Melphalan
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Which alkylating agents are used to treat lymphoma?
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Procarbazine
Dacarbazine |
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What is the test-writers' favorite alykylating agent?
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Chlorambucil
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Which drugs are microtuble inhibitors?
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Vincristine, Vinblastine - inhibit formation of microtubules
Paclitaxel - locks up microtubules after they have formed |
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Which chemo drug is an immune modulator?
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Levamisole - stimulates NK cells
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Which protein is the only one to be completely modified in the Rough ER?
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Collagen
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What are the 2 mitochondrial diseases? How are they inherited? What is the clue?
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Leber's - blind w/in few months of life
Leigh's - chronic fatigue Mom--> all her children Clue: Ragged red fibers |
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If no signal for enzymes to go to lysosome, what disease results?
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I-cell disease: inclusion bodies
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What are the 6 enzyme deficiencies that are X-linked recessive?
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Fabry's - alpha-galactosidase
Lesch Nyhan - HGPRT Hunter's - iduronidase CGD - NADPH Oxidase G6PD Adrenoleukodystrophy - carnitine acetyltransferase I |
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What nucleotide is most sensitive to UV light?
What enzyme fixes mutations that occur 2/2 UV light exposure? What happens if this enzyme is deficient? |
Thymidine
UV light endonuclease Partial deficiency: lizard skin/ icthyma Complete deficiency: xeroderma pigmentosa |
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What four mechanisms does our body use to store DNA in euchromatin
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1) methylation
2) acetylation of histones 3) add G's and C's to parts of genome 4) Use repressor proteins to inhibit transcription |
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What two types of mutations can lead to differentiation between early/late syndromes?
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1) Repressor proteins - if mutated early = early onset; if mutated late = late onset
2) Nonsense (early) vs. Missense (late) mutations |
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If I don't know the mutation that causes a cancer, choose...
How does this mutation work? |
p53 - responsible for 60% of cancers
D-cyclin is defective and can't direct cells toward apoptosis --> cancer |
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If I see a baby with gout, the disease is due to a deficiency of...
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HGPRT
If complete deficiency = Lesch Nyhan Syndrome |
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What is the pathophysiology that causes SCID?
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Adenosine deaminase is deficient
Adenosine deaminase is responsible for regulating ribonucleoside reductase which is needed to make DNA -->def. affects BM first |
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What disease state presents with anti-histone antibodies?
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Drug-Induced Lupus
Hydralazine, INH, Penacillamine, Procainamide, Phenytoin, Ethosuximide |
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Which disease presents with anti-topoisomerase antibody?
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Progressive Systemic Sclerosis - worst type of scleroderma
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Which disease presents with anti-SnRNP antibodies?
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mixed connective tissue diseases
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