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MCB1 Block 3 > Lysosome > Flashcards

Lysosome Flashcards

1
Q

What is significant about the lysosome membrane?

A

It is heavily glycosylated to protect the lysosome from degrading itself

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2
Q

What are lysosomes filled with?

A

acid hydrolases

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3
Q

What is the pH in a lysosome?

A

4.5-5

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4
Q

Proteases, nucleases, lipases, phospholipases, phosphatases, sulfatases are all examples of what?

A

acid hydrolases (what fill lysosome)

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5
Q

What is a primary lysosome?

A

newly synthesized with acid hydrolases but have not yet aquired material or degraded anything

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6
Q

What is a secondary lysosome?

A

a lysosome that has or is degrading something

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7
Q

What is TGN?

A

trans golgi network

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8
Q

Primary lysosomes bud from what?

A

trans golgi network

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9
Q

If a vesicle is going from golgi to endosome what is the only thing it carries?

A

lysosomal enzymes

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10
Q

What goes from an endosomeo to golgi?

A

membrane and M6P receptors

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11
Q

how does an endosome become a lysosome?

A

Combination of fusing and lowering of pH due to proton pumps

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12
Q

Where do vesicles that contain material to be degraded go once they enter cell via endocytosis?

A

They go to early endosome then to late endosome then to lysosome (the endosome becomes these things)

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13
Q

After a vesicle leaves the TGN, what are its three options?

A
  1. Constitutive secretion
  2. Regulated secretion
  3. Lysosomal/endosomal pathways
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14
Q

When a vesicle has a clathrin coat, where is it going?

A

lysosome or endosome

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15
Q

What is the purpose of GlcNAc phosphotransferase

A

It puts the M6P tag on the protein in the cis Golgi

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16
Q

What does M6P tag have a high affinity for?

A

M6P receptor in TGN

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17
Q

What puts the sugar chains on the protein in the rER?

A

Dolichol

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18
Q

What is the function of dolichol?

A

puts on n-linked oligosaccharide chains on in rER

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19
Q

Where is the only location clathrin will coat vesicle?

A

From golgi to lysosome or endosome, and from plasma membrane into a cell.

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20
Q

When a vesicle coated in clathrin buds off, what allows the final “budding” or “pinching” off of the vesicle from the membrane?

A

dynamin GTPase

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21
Q

When will COPII form?

A

vesicle going from ER to golgi

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22
Q

When will COPI form?

A

vesicle going from golgi to ER

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23
Q

How do hydrolases get into lysosome?

A 
Cargo binds M6P receptor
M6P receptor clusters →adaptors →clathrin
vesicle buds off
clathrin removed
fusion with endosome
acidic enviornment → M6P receptor dissociates
Phosphate removed from cargo
M6P recycled back to TGN
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24
Q

Why does phosphate need to be removed from cargo?

A

So it cannot bind back to M6P receptor

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25
Q

What is the name of the pump that pumps H+ into lysosome?

A

V-type ATPase

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26
Q

If lysosome fuses with extracellular membrane, where would the glycosylation face?

A

extracellular space (in the lysosome, they face the lumen)

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27
Q

What are the 3 pathways to degradation in lysosome?

A

Endocytosis
Phagocytosis
Autophagy

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28
Q

Phagosomes fuse with what?

A

lysosome

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29
Q

What is autophagy?

A

the cell is digesting something w/in itself

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30
Q

What do organelles (EX: mitochondria) that are not needed or are too old in a cell do to be degraded?

A

They form autophagasome and fuse with lysosome

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31
Q

What is a multivesicular body?

A

an intermediate step b/w early endosome and late endosome

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32
Q

If you want to down regulate a receptor because a cell has enough of a substance, how would you degrade a transmembrane protein?

A

multivesicular body - the membrane fuses together and creates areas inside the cell where the transmembrane protein can be, which will eventually be degraded

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33
Q

How does the lysosome/endosome know when a receptor is no longer needed and needs to be degraded?

A

it will be mono-ubiquinated

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34
Q

What is mono-ubiquitin tag?

A

It downregulates receptors/proteins

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35
Q

What is the product when a phagosome joins with a lysosome and digests the material?

A

Residual body & indigestible material

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36
Q

If product accumulates in cytosol after lysosome and phagosome degrade, what is it called?

A

lipofucin

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37
Q

Describe what happens in autophagy

A

ER envelopes old organelle, then fuses with a lysosome

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38
Q

What envelopes old organelles for autophagy?

A

ER

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39
Q

If lysosome can’t degrade material within it, it becomes a ______

A

residual body

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40
Q

What happens to residual body?

A

it is expelled from cell or remains in cell as lipofuscin

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41
Q

Pigmented lipids that accumulate in organs is what?

A

lipofuscin

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42
Q

What is the mode of inheritance for lysosomal storage diseases?

43
Q

Most lysosomal storage diseases affect what?

A

individual hydrolase

44
Q

What is the most sever lysosomal storage disease?

A

I-cell disease

45
Q

Explain the gist of a lysosomal storage disease

A

hydolase doesn’t work correctly →accumulation in lipid →enlarged lysosomes →inhibits function of cell

46
Q

Tay Sachs is what kind of disease?

A

lysosomal storage

47
Q

Gaucher is what kind of disease?

A

lysosomal storage

48
Q

What are gags?

A

glycosaminoglycans (basically sulfated sugars)

49
Q

Mucopolysaccharidoses is what kind of disease?

A

lysosomal storage

50
Q

Metachromatic leukodystrophy is what kind of disease?

A

lysosomal storage

51
Q

pseudo-Hurler polydystrophy is what kind of disease?

A

lysosomal storage

52
Q

Mucopolysaccharidoses has the accumulation of what?

53
Q

Metachromatic leukodystrophy has the accumulation of what?

A

sulfatides

54
Q

pseudo-Hurler polydystrophy is similar to what disease?

A

I-cell, but not as severe

55
Q

key words: absence of M6P, waste products in inclusion bodies, coarse facial features, enlarged liver/spleen, death CHF are what disease?

56
Q

What is the mechanism for I-cell?

A

Acid hydrolases lack H6P so are secreted extracellulary, build up of stuff in lysosome

57
Q

These characteristics: later onset than I Cell, survival into adulthood are linked to what disease?

A

Pseudo-Hurler Polydystrophy (MUCOLIPIDOSIS III)

58
Q

what is Hirsutism?

A

excessive facial hair

59
Q

Characteristics: alpha-L-iduronidase, dermatan sulphate & heparan sulphate, hepatosplenomegaly, coarse facial features, skeletal deformity, hirsutism, corneal clouding are what disease?

A

Hurler Syndrome

60
Q

MPS IH stands for what?

A

Hurler syndrome

61
Q 
Symptoms: Physical & mental deterioration
Growth stops at 2-4 yrs
Hepatosplenomegaly
Deafness
Skeletal deformity
Coarse facial features
Hirsutism
Thickened skin
Corneal clouding
at a few months old is what disease?
A

Hurler syndrome

62
Q

Which disease has a deficiency of α-L-iduronidase ?

A

Hurler syndrome

63
Q

Deficiency of α-L-iduronidase causes what?

A

accumulation of dermatan sulphate & heparan sulphate

64
Q

accumulation of dermatan sulphate & heparan sulphate is what disease?

A

hurler syndrome

65
Q

What are dermatan sulphate & heparan sulphate ?

A

2 kinds of GAGs

66
Q

Characteristics: Milder form of Hurler Syndrome are what disease?

A

Scheie & Hurler-Scheie Syndrome

67
Q

What disease has residual α-L-iduronidase activity?

A

Scheie & Hurler-Scheie syndrome

68
Q

MPS IS & MPS IHS stands for what?

A

Scheie & Hurler-Scheie syndrome

69
Q

MPS IS is what?

A

Schaeie syndrome

70
Q

MPS IHS is what?

A

Hurler-scheie syndrome

71
Q

Of the MPS diseases, rank from most severe to least severe

A

IH - IHS - IS

72
Q

Characteristics: later presentation (2-4 years old) and milder course, surviving into mid 30s, no corneal clouding is what disease?

A

Hunter syndrome

73
Q

Key words: iduronodate sulphatase, no corneal clouding is what disease?

A

Hunter syndrome

74
Q

MPS II is what disease?

A

Hunter syndrome

75
Q

What is the mode of inheritance for hunter syndrome?

A

x-linked recessive

76
Q

how is the mod of inheritance different in hunter syndrom from other lysosomal storage syndromes?

A

Most are autosomal recessive, hunter is x-linked recessive

77
Q

Deficiency of enzyme iduronodate sulphatase is what disease?

A

Hunter syndrome

78
Q

Hunter syndrome has deficiency of what enzyme?

A

iduronodate sulphatase

79
Q

dermatan sulphate & heparan sulphate are examples of what?

80
Q

What is the mechanism for Hunter syndrome?

A

Deficient iduronodate sulphatase ! accumulation of dermatan sulphate & heparan sulphate (GAGs)

81
Q

What kind of disease is Sanfilippo syndrome ?

A

Lysosomal storage

82
Q

MPS III stands for what?

A

Sanfilippo syndrome

83
Q

Key words: short, pectus carinatum, normal IQ; Often mistaken for skeletal dysplasia rather than lysosomal storage disease are what disease?

A

Morquio Syndrome (MPS IV)

84
Q

Symptoms: normal IQ, short stature, pectus carinatum (pigeon chest), aortic regurgitation ! cardiomegaly, deafness, weakness are what disease?

A

Morquio Syndrome (MPS IV)

85
Q

What disease has Defective degradation of keratan sulphate

A

Morquio Syndrome (MPS IV)

86
Q

What is MPS IV?

A

Morquio Syndrome

87
Q

What lysosomal storage disease is often misdiagnosed as skeletal dysplasia?

A

Morquio syndrome

88
Q

What is the mechanism of Morquio syndrome?

A

accumulatino of GAGs due to defective degradation of keratan sulphate

89
Q

Characteristics: Similar to Hurler with normal IQ are what disease?

A

Maroteaux-Lamy Sydrome (MPS VI)

90
Q

MPS VI is what disease?

A

Maroteaux-Lamy Sydrome

91
Q

MPS VII is what disease?

A

Sly syndrome

92
Q

What disease has deficient ARSA (Arylsulfatase A)?

A

Metachromatic Leukodystrophy

93
Q

What kind of disease is Metachromatic Leukodystrophy?

A

lysosomal storage

94
Q

What is the mechanism for Metachromatic Leukodystrophy?

A

Deficient ARSA → defective degradation of sulfatides → accumulation of sulfatides in cells → progressive leukodystrophy in CNS & PNS → paralysis & cognitive decline

95
Q

How severe is Metachromatic Leukodystrophy?

A

Depends on age of onset, three different forms

96
Q

Key words: CHS1/LYST, phagocytosis, autophagocytosis, recurrent infections are what disease?

A

Chediak-Higashi Syndrome

97
Q

Recurrent infections (life threatening), hypopigmentation, mild coagulation defects, varying neurological problems; Treatment = BMT (hematologic & immune defects) is what disease?

A

Chediak-Higashi Syndrome

98
Q

What is CHS1/LYST?

A

lysosomal trafficking regulatory protein involved in vesicle fusion). Know that it is involved in vesicle fusion

99
Q

What kind of disease is Chédiak-Higashi syndrome

?

A

Not lysosomal storage - but related to lysosomal function

100
Q

What is the mechanism for Chediak-Higashi syndrome?

A

Mutated CHS1/LYST: delayed fusion of phagosome with lysosome in leukocytes & granular defects in NK cells →recurrent infections; granular defects in platelets →coag. defects; autophagocytosis of melanosomes in melanocytes → albinism

101
Q

What membrane is similar to lysosomal membrane?

A

Melanocytes

102
Q

What are NK cells?

A

natural killer

103
Q

Besides Hurler, what other disease has the accumulation of dermatan sulphate & heparan sulphate?

A

Hunter Syndrome

MCB1 Block 3 (15 decks)

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