Protein/Endo Trafficking Flashcards

1
Q

Three domains of the endoplasmic reticulum

A

1) Smooth ER
2) Rough ER
3) ERGIC

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2
Q

What is the characteristic function of the smooth ER?

A

lipid metabolism

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3
Q

What is the characteristic aspect of the rough ER?

A

covered by ribosomes

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4
Q

What does ERGIC stand for?

A

ER-Golgi Intermediate Complex

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5
Q

How is the golgi apparatus’ structure characterized?

A

stacked and polarized cisternae

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6
Q

Where does the cis face of the golgi apparatus face?

A

Endoplasmic reticulum

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7
Q

What does topology of the secretory pathway refer to?

A

Shit that is facing the cytosol continues to do so throughout the various fusions, etc.

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8
Q

Protein synthesis by free cytosolic ribosomes leads to what type of translocation?

A

Post-Translational Translocation

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9
Q

Protein synthesis by membrane-bound ribosomes leads to what type of translocation?

A

Co-Translational Translocation

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10
Q

Transmembrane proteins must use which method of translocation?

A

Co-translational translocation

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11
Q

What binds the signal sequence translated first in the beginning of co-translational translocation?

A

Signal Recognition Particle (SRP). Brings it into the SRP receptor on the ER membrane.

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12
Q

What does the ribosome bind to on the ER membrane in co-translational translocation?

A

Translocon

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13
Q

Which enzyme cleaves the signal sequence of the growing polypeptide in co-translational translocation?

A

Signal peptidase

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14
Q

What are the heat shock proteins involved in post-translational translocation?

A

Hsp 70 and Hsp 40. They maintain the protein in an unfolded state.

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15
Q

What recognizes the signal sequence in post-translational translocation

A

Sec62/63

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16
Q

What is the name of the ER chaperone which holds proteins unfolded until the whole thing passes through in post-translational translocation

A

BiP

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17
Q

An oligosaccharide synthesized on a lipid carrier. Prevents protein aggregation in the ER, which helps sort signals.

A

N-linked glycosylation

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18
Q

Which terminus are glycolipids added on to create a GPI anchor and attach proteins to the membrane?

A

C-terminus

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19
Q

What is the cytosol-side peptide signal for transmembrane proteins?

A

2 methionine

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20
Q

What is the cytosol-side peptide signal for lumenal proteins?

A

2 glutamic acid

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21
Q

What is the cytosol-side peptide signal for GPI-anchored protein?

A

2 aspartic acid

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22
Q

Specialized region of the ER where proteins and phospholipids are exported in vesicles

A

ER Exit Site (ERES)

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23
Q

What makes up the signals that direct proteins packaged for vesicle transport?

A

peptide and carbohydrate signals

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24
Q

Unmarked proteins in the ER can also be packaged and transported where by a “default pathway”?

A

Golgi

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25
Q

Proteins destined for retrieval by the endoplasmic reticulum have which two targeting sequences at their C termini?

A

KDEL or KKXX (there’s then a KDEL receptor)

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26
Q

What two important membrane components are synthesized mostly in the golgi apparatus?

A

Glycolipids and sphingomyelin

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27
Q

Four regions of the Golgi apparatus

A

1) cis compartment
2-3) medial and trans compartments
4) trans golgi network

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28
Q

Region of the golgi apparatus which receives molecules from the ERGIC

A

cis compartment

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29
Q

Region of the golgi apparatus which is responsible for most modifications

A

medial and trans compartments

30
Q

Region of the golgi apparatus which is the sorting and distribution center

A

trans-golgi network

31
Q

Golgi protein movement theory in which transport vesicles carry proteins between cisternae

A

stable cistarnae model

32
Q

Golgi protein movement theory in which proteins are carried within the cisternae, which gradually mature and progressively move through the golgi in the cis to trans direction

A

cisternal maturation model

33
Q

Which carbohydrates, added to proteins in the ER, are modified in the golgi?

A

N-linked oligosaccharides

34
Q

In the golgi apparatus, what can mannoses be swapped out for?

A

1) NAG
2) galactose
3) sialic acids

35
Q

What sugar can be added to the origin N-acetylglucosamine in the Golgi apparatus?

A

Fucose

36
Q

What phosphorylated sugar signals for proteins to go to the lysosome in the trans golgi network?

A

mannose-6-phosphate

37
Q

Carbohydrates added to the side chains of serine and threonine, creating proteoglycans, are called….

A

O-linked glycosylation

38
Q

Three routes to go from the Golgi to the cell surface

A

1) direct transport to the plasma membrane
2) recycling endosomes
3) regulated secretory pathways

39
Q

What sort of vesicle are hormones and neurotransmitters packaged in the trans-Golgi for regulated release

A

secretory granules

40
Q

The three types of vesicle coat proteins

A

1) COPII-coated vesicles
2) COPI-coated vesicles
3) Clathrin-coated vesicles

41
Q

Which family of vesicle coat proteins carries proteins from the ER to the ERGIC and on to the golgi apparatus?

A

COPII-coated vesicles

42
Q

Which family of vesicle coat proteins bud from the ERGIC or Golgi and carry their cargo back, returning proteins to earlier compartments

A

COP1-coated vesicles

43
Q

Which family of vesicle coat proteins transport in both directions between the trans Golgi network, endosomes, lysosomes, and the plasma membrane

A

Clathrin-coated vesicles

44
Q

mnemonic: “two steps forward, one step back”

A
COPII = anterograde transport
COPI = retrograde transport
45
Q

Which small GTP-bind proteins, related to Ras and Ran, regulate the formation of coated vesicles

A

ARF1 and Sar1

46
Q

What two things mediate interactions between transport vesicles and target membranes?

A

tethering factors and Rab proteins (small-GTP binding proteins)

47
Q

What protein on the vesicle drives fusion?

A

vSNARES

48
Q

What protein on the target membrane drives fusion?

A

tSNARES

49
Q

What is the motor protein involved in membrane fusion?

A

Dynamin

50
Q

What does Rab bind on target membranes?

A

Tethering factors

51
Q

What binding motif do SNARE proteins have?

A

central coiled-coil domain

52
Q

What is the ideal pH for most of the lysosomal enzymes which are called “acid hydrolases”

A

5

53
Q

How is a lysosome formed?

A

A transport vesicle fuses with a late endosome

54
Q

They fuse with endocytic vesicles from the plasma membrane in order to separate molecule for recycling from molecules destined for degradation in lysosomes.

A

Early endosome

55
Q

Molecules to be recycled are passed to these and back to the plasma membrane

A

Recycling endosomes

56
Q

Carry molecules to be degraded by lysosomes

A

Late endosomes

57
Q

Phagosomes fuse with lysosomes to become…

A

phagolysosomes

58
Q

Mechanism for selective uptake of specific macromolecules

A

Clathrin-mediated endocytosis

59
Q

Specialized region where macromolecules can bind to cell surface receptors

A

clathrin-coated pits

60
Q

Endocytosed (is that a fucking word) clathrin-coated vesicles then fuse with what?

A

early endosomes

61
Q

What causes receptors and ligands to separate in early endosomes?

A

acidic pH (6.0 - 6.2)

62
Q

Examples of clathrin-independent endocytosis (3)

A

1) macropinocytosis
2) internalization of caeolae
3) uptake of GPI-anchored plasma membrane proteins clustered in lipid rafts

63
Q

What does LDL stand for

A

Low-density lipoprotein

64
Q

LDL / clathrin coated pit dysfunction where either the receptors don’t bind LDL or the mutations prevent the receptors from concentrating in the coated pits

A

Familial hypercholesterolemia

65
Q

The six amino acid signal, including tyrosine, which prevents LDL receptors from concentrating in coated is called what

A

internalization signal

66
Q

What does CEDNIK syndrome stand for?

A

Cerebral dysgenesis, neuropathy, ichthyosis, and palmoplantar keratoderma

67
Q

What is the inheritance pattern of CEDNIK syndrome?

A

autosomal recessive

68
Q

What is mutated in CEDNIK syndrome?

A

SNAP29 (part of the SNARE complex)

69
Q

I-Cell Disease is also referred to as…

A

mucolipidosis II

70
Q

Which gene is mutated in I-cell disease?

A

GNPTAB (chromosome 12)

71
Q

GNPTAB gene (well the protein it codes) is the first step in what process?

A

tagging lysosomal enzymes with mannose-6-phoasphate in the golgi apparatus

72
Q

Coarse facial features, joint limitations, skeletal abnormalities, cardiomegaly, developmental delay, and early loss of life…

A

I-cell disease