Lysosomes Flashcards

1
Q

Where do ribosomes start translation? [1 mark]

A

At the inititation sequence (AUG)

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2
Q

What is the signal sequence and what is its purpose? [4 marks]

A
  • 10-30 amino acid long sequence
  • Rich in hydrophobic amino acids
  • Guides ribosome complex to the ER surface
  • Causes ribosome to be docked to the ER membrane
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3
Q

What does the signal recognition particle (SRP) do? [3 marks]

A
  • Binds to the signal sequence and large ribosomal unit
  • Stalls translation
  • Guides ribesome to translocon
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4
Q

What happens once ribosome reaches the translocon [4 marks]

A
  • Protein is threaded through the translocon into the lumen of ER
  • Protein folds inside
  • GTP is hydrolysed to provide energy for processes
  • Then proteins are transported through vesicle into Golgi apparatus (cis to trans) then to lysosomes
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5
Q

Where are lysosomal enzymes made? [1 mark]

A

Endoplasmic reticulum

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6
Q

Structure of lysosome [3 marks]

A
  • Single glycosylated membrane with lots of transmembrane proteins
  • Heteromorphic
  • Low pH
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7
Q

Substrate delivery to lysosomes for EXTERNAL substrates [2 marks]

A
  • Fluid-phase endocytosis

- Phagocytosis

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8
Q

What does fluid phase mean? [2 marks]

A
  • Fluids from outside are taken up

- Molecules destined for digestion are dissolved in the fluid

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9
Q

Endocytosis process for digestion [5 marks]

A

Pit in plasma membrane → pinches off to form vesicle → early endosome → late endosome → fuses with lysosome

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10
Q

Phagocytosis process [3 marks]

A

Particle engulfed by plasma membrane → phagosome formed → phagosome fuses with lysosome

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11
Q

What are the differences between phagocytosis and autphagy? [4 marks]

A
  • Single membrane surrounds substrate in phagocytosis
  • Double membrane for autophagy
  • Substrate to undergo phagocytosis is wrapped in plasma membrane.
  • For autphagy, ER membrane seals it off
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12
Q

Substrate delivery to lysosomes for INTERNAL substrates [1 mark]

A
  • Autophagy
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13
Q

Receptor mediated endocytosis for LDL [4 mark]

A
  • LDL binds to its receptor and forms a complex
  • Complexes aggregate in coated pits and pinch off (forming a vesicle)
  • pH drops and receptors dissociate
  • Cholesteryl ester is hydrolysed with lipase (forms cholesterol and fatty acids)
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14
Q

What is glycosylation of lysosomal enzymes and why is it important? [3 marks]

A
  • When lysosomal enzymes receive oligosaccharides into Asp residues
  • Singal sequence cleaved off by signal peptidase
  • Important as without it, some proteins can’t fold correctly
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15
Q

Mannose-6-phosphate pathway [2 marks]

A

M6P attaches to lysosomal enzyme → Vesicles form around modififed enzyme

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16
Q

What is clathrin and why is it important? [2 marks]

A
  • Protein that plays an important role in forming coated vesicles
  • Leaves vesicles once they’ve been formed
17
Q

Default pathway [1 mark]

A

Proteins without M6P leave cell via exocytosis

18
Q

M6P receptors at plasma membrane [1 mark]

A

Recaptures modified proteins that have taken the default pathway

19
Q

I cell cause [3 marks]

A
  • Mutation in enzymes needed for M6P modification
  • Deficiency in GlcNAc phosphotransferase
  • Lysosomal enzymes take the default root
20
Q

I cell effects in tissue [3 mark]

A
  • Substates for lysosomal enzymes build up
  • Dense inclusion (lysosomes without) build up, dense with substates
  • Particularly affects macrophages, neutrophils, microglia and hepatocytes
21
Q

I cell symptoms

A
  • Skeletal abnormalities
  • Developmental delay
  • Enlarged liver and spleen
  • Impaired hearing
  • Early death