lysosomes and proteosomes Flashcards

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1
Q

de Duve

A

discovered lysosomes through differential gradient centrifugation
- “injured” (homogenized) = hydrolytic
- lysosomes co-sedimented with mitochondria

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2
Q

lysosomes

A

a) 50+ hydrolytic enzymes

  • degrade nearly all molecules
  • e.g nucleases, phosphatases, proteases

b) single membrane bound
c) pH 5 = optimal

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3
Q

acid phosphatase

A

marker enzyme for lysosomal identification

  • cleaves phosphates from proteins
  • works best in acidic environments
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4
Q

heterophagy

A
  • most closely associated with lysosomes
  • Rudolph Virchow - looked at pus, thought WBC were giving birth to RBC
  • Elie Metchnikoff - phagocytosis, discovered WBCs prevent disease

cell consumes (eats) material

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5
Q

opsinization

A

antibodies tag a bacterium and the Fc region of the antibody binds to the Fc receptor for phagocytosis

ex: leukocytes have these receptors

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6
Q

Myasthenia Gravis

A
  • autoimmune disease
  • acetylcholine receptors are internalized and degraded = impaired muscle contraction
  • antibodies form against nicotinic acetylcholine synapse receptors
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7
Q

curare

A

plant poison

  • antagonist of nicotine Ach receptor
  • blocks Ach receptor
  • increased symptoms

drug = vyvgart

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8
Q

eserine

A
  • inhibits breakdown of acetylcholinesterase
  • relieved symptoms in patients
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9
Q

Torpedo Rays

A
  • electroplax
  • used to generate anti-Ach antobodies
  • highest code of Ach-R
  • when injected into mice, caused droopy eyelids
  • indicated MG may be an autoimmune disease
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10
Q

3H bungarotoxin

A
  • binds to Ach-R
  • showed their were fewer number of receptors on postsynaptic component of neuromuscular junction
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11
Q

autophagy

A
  • self digestion and has to do with cell death
  • mitophagy –> aging
  • LC3 mediates autophagy
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12
Q

chloroquine

A

autophagy lysosomal drug

  • increases pH of lysosome which decreases its activity
  • used to sensitize cancer cells to chemo drugs (cisplatin), but harms normal cells too
  • cisplatin blocks nuclear DNA repair process –> apoptosis
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13
Q

specialized lysosomes

A

acrosome + plant vacuole

plant vacuoles serve as “lysosomes”

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14
Q

Silicosis (Miner’s disease)

A
  • lungs become less elastic because of collagen deposition
  • silica particles are sharp
  • they enter the lungs and lysosomes, causing leak of hydrolytic enzymes = cells die

fibrosis : collagen secreted due to an injury
- decreases CO2/O2 exchange and elasticity of the lungs

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15
Q

chloroquine retinopathy/myopathy

A
  • drug induced lysosomal disease
  • not heritable
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16
Q

inherited lysosomal diseases

A
  • 70 different types of storage diseases
  • treated with enzyme replacement therapy
17
Q

Tay Sachs Disease

A

inherited lysosomal disease

  • Hex A deficiency
  • can’t break down ganglioside GM2 –> accumulates in the brain
18
Q

Hurlers Syndrome (Gargoylism)

A

inherited

  • cis golgi
  • gangliosides accumulate –> large lysosomes
  • “co-culture” experiment “conditioned medium”
  • Hurler cells look normal when put in culture with normal cells
  • drug = Aldurazyme (ERT)
19
Q

I cell disease

A

inherited

  • cis golgi
  • lots of hydrolases in the extracellular medium
  • I cells can endocytose - M6P is ok
  • I cell lysosomal enzymes can’t be endocytosed in normal fibroblast - M6P tag wasn’t added
20
Q

Pompe’s Disease

A
  • cis golgi
  • defect in acid alpha glucosidase
  • buildup of glycogen
  • muscular generative disease
  • myozyme = ERT drug
20
Q

Gaucher’s Disease

A

inherited

  • cis golgi
  • lysosomal disease
  • deficiency in glucocerebrosidase
  • leads to enlargement of spleen and erosion of the long bones
  • cerezyme - ERT drug
20
Q

lysosomes

A
  1. degrades all molecules
  2. is an organelle
21
Q

proteasomes

A
  1. degrades only proteins
  2. “particles” - “machine”
  3. large (750,000 Daltons)
    - 30,000 per cell
  4. target for cancer
  5. Velcade - blocks proteasomes and inhibits protease activity
  6. ubiquinated
  7. mutant yeast without proteasomes –> die
22
Q

velcade

A

proteasome drug that targets cancer