Lysosomes Flashcards

1
Q

where are lysosomes made?

A

in the ER

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2
Q

what is the pH inside the lysosome

A

pH 4.5

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3
Q

why is the pH low in the lysosomes?

A
  • enzymes require low pH for optimum activity

- to regulate enzyme functions: if lysosomes were to break, enzyme will degrade in the cytoplasm due to high pH

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4
Q

describe the structure of lysosomes

A
  • single membrane
  • acidic
  • heterogenous contents
  • 100 lysosomes per cell
  • 40 hydrolytic enzymes
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5
Q

what 2 ways do substrates enter a lysosome?

A
  • extracellular substrates: endocytosis

- intracellular: microautophagy

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6
Q

give an example of when substrates would enter the lysosome by endocytosis

A

-phagocytosis

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7
Q

how do low density lipoproteins (LDLs) enter the lysosome?

A
  • LDL is the main transport vehicle of cholesterol in bloodstream
  • LDL binds to receptors on membrane
  • receptors aggregate to form coated pits
  • the pit folds inwards and the membrane detaches itself to form a closed coated vesicle.
  • pH drops in vesicle so LDL dissociates from receptor and receptor returns to membrane
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8
Q

what is LDL hydrolysed into?

A

cholesteryl ester and fatty acid by lipase enzymes

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9
Q

what modification do lysosomal enzymes have that others don’t

A

mannose-6-phosphate

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10
Q

what is microautophagy?

A

when the lysosome directly enfgulfs the target particle or organelle

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11
Q

what is macroautophagy?

A

when cells form vesicles called autophagosomes and fuse with lysosomes to degrade cell contents

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12
Q

what is the name of the enzyme that adds the M6P modification

A

phosphoryl transferase

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13
Q

where do proteins that do not have the M6P modifications go

A

plasma membrane

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14
Q

what happens if there is no M6P modification

A

the enzyme gets diverted and is secreted, substrate builds up inside the lysosome.

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15
Q

describe the mannose 6-phosphate (M6P) pathway

A
  • digestive enzymes & membrane proteins of the lysosome are synthesised in the ER & transported through the Golgi apparatus to the trans-Golgi network.
  • while in the ER & cis-Golgi network, the enzymes are tagged with a specific phosphorylated sugar group (mannose 6-phosphate).
  • when they arrive in the trans-Golgi network they can be recognized by an appropriate receptor, the mannose 6-phosphate receptor.
  • the tagging permits the enzymes to be sorted & packaged into transport vesicles, which bud off & deliver their contents to the lysosome via late endosomes.
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16
Q

name the disease when M6P is not produced and it’s symptoms

A
  • I cell disease: M6P not produced so enzymes take default pathway and secreted out of cell and not transported to lysosome

symptoms:

  • skeletal abnormalities
  • developmental delays
  • enlarged liver and spleen
  • impaired hearing
  • death within first decade
  • may lead to stuttering