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M2M Unit IV > Cilia and Ciliopathies > Flashcards

Cilia and Ciliopathies Flashcards

(71 cards)

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1
Q

The distinguishing factor between motile and immotile cilia is the presence of ________.

A

axonemal dynein arms between the doublet microtubules

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2
Q

The ciliary membrane is continuous with the cellular _____.

A

plasma membrane

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2
Q

How many genes are known to be mutated in BBS?

A

19

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4
Q

An estimated _____proteins are coordinated to form the cilium.

A

1,000

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4
Q

Microphthalmia

A

Small eye developmental disorder

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5
Q

The ______ is the structural skeleton of the cilium.

A

axoneme

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5
Q

Nephronophthisis

A

Progressive renal damage

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5
Q

Polydactyly

A

extra digit on the ulnar side of hand or foot

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6
Q

Cilia can sense ____ and ____ stimuli.

A

physical; chemical

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6
Q

situs inversus

A

Left-right laterality defects to randomize organ position

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6
Q

How is PKD inherited?

A

both autosomal dominant and recessive forms

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7
Q

What is anterograde transport?

A

movement towards the ciliary tip

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9
Q

The transition zone links the ____ to the ____ and to the ______.

A

basal body; axoneme; ciliary membrane

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9
Q

______, ________, or _________ possess a 9+0 microtubule arrangement.

A

Non-motile, sensory or primary cilia

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10
Q

Ciliogenesis normally occurs during ____ phase of the cell cycle.

A

G1 (or G0)

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12
Q

The basal body is formed beginning at the _____ and the distal-end is responsible for _____.

A

basal body; nucleating the cilium

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13
Q

_____ is caused by mutations in polycystin-1 and polycystin-2.

A

ADPKD

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14
Q

How does retrograde transport occur?

A

by cytoplasmic dynein 2 motor driven transport with the IFT-A protein complex

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14
Q

What establishes left-right asymmetry of the lateral body plane?

A

the ciliary node

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14
Q

What does the beating of the ciliary node cilia do?

A

produces a net leftward flow of signaling molecules/morphogens

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15
Q

Where does ciliogenesis begin?

A

at the distal end of the basal body

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16
Q

Which centriole becomes the basal body?

A

the older one (the mother centriole?

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17
Q

ADPKD is caused by mutations in _____ and _____.

A

polycystin-1; polycystin-2

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18
Q

What is the purpose of the ciliary node?

A

establishes left-right asymmetry of the lateral body plane

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19
The ______ is formed beginning at the proximal-end and the distal-end is responsible for nucleating the cilium.
basal body
20
\_\_\_\_\_ is caused by fibrocystin mutations.
ARPKD
22
The _____ is formed from doublet microtubules (A-B tubules) that assemble from the A- and B-tubules of the basal body.
axoneme
23
Non-motile, sensory or primary cilia possess a \_\_\_\_\_\_\_.
9+0 microtubule arrangement
24
How is Bardet-Biedl Syndome (BBS) inherited?
autosomal recessive
25
The hedgehog (Hh) signaling pathway is well established to signal through \_\_\_\_.
cilia
26
The ______ links the basal body to the axoneme and to the ciliary membrane.
transition zone
27
\_\_\_\_\_ normally occurs during G1 (or G0).
Ciliogenesis
28
The _____ is continuous with the cellular plasma membrane.
ciliary membrane
29
What does BBS stand for?
Bardet-Biedl Syndome
30
The _____ signaling pathway is well established to signal through cilia.
hedgehog (Hh)
31
The axoneme is formed from ______ that assemble from the\_\_\_\_\_\_\_ of the basal body.
doublet microtubules (A-B tubules); A- and B-tubules
31
What does PKD stand for?
Polycystic Kidney Disease
32
What is intraflagellar transport (IFT)?
bidirectional transport with kinesin motors and the IFT-B protein complex directing movement to the ciliary tip
33
Motile cilia typically have a _____ arrangement.
9+2 microtubule
34
What is the incidence rate of ciliopathies?
1:100,000
35
What is the incidence rate of ADPKD?
1:1000
37
Basal bodies are _____ cylinder shaped structures formed from nine triplet microtubules
microtubule rich
38
Occipital meningoencephalocele
Neural tube defects with hernial protrusion of the brain material
39
How many genes are known to be mutated in ciliopathies?
50
40
Lung hypoplasia
Incomplete lung development
41
BBS proteins participate in a protein complex that is required for _____ within the cilium.
vesicle transport
42
Major cilia domains include the centriole/basal body, \_\_\_\_\_, transition zone, \_\_\_\_\_\_, and intraflagellar transport (IFT) machinery.
axoneme; ciliary membrane
43
Renal hypodysplasia or dysplasia
Abnormal or missing kidney formation
45
\_\_\_\_\_ typically have a 9+2 microtubule arrangement.
Motile cilia
47
Activation and repression of the target of the _____ requires cilia.
Hh paracrine signaling pathway
48
Major cilia domains include the \_\_\_\_\_\_, axoneme, \_\_\_\_\_\_, ciliary membrane, and \_\_\_\_\_.
centriole/basal body; transition zone; intraflagellar transport (IFT) machinery
49
Basal bodies are derived from \_\_\_\_\_\_.
centrioles
50
ARPKD is caused by ______ mutations.
fibrocystin
52
\_\_\_\_\_ are the core anchors from which cilia are formed.
Basal bodies
53
Amnosia
Loss in olfaction
54
\_\_\_\_ are microtubule rich cylinder shaped structures formed from nine triplet microtubules
Basal bodies
56
\_\_\_\_\_\_ provide the tracks for movement within cilia.
Axonemes
57
How is cargo transported along the axoneme?
intraflagellar transport (IFT)
59
Cystic kidneys
Multilumen formation and growth of fluid filled cysts
60
What is required for cilium formation and function?
anterograde and retrograde intraflagellar transport
61
Why is the cilia used for cell signaling?
the cilium concentrates the signal with a high receptor surface to volume ratio
62
Activation and repression of the target of the Hh paracrine signaling pathway requires \_\_\_\_\_.
cilia
63
Cilium lengths range from _____ to \_\_\_\_\_\_.
less than a micron; tens of microns
64
What is the incidence rate of ARPKD?
1:20,000
65
Centriole duplication occurs during the _____ phase of the cell cycle.
G1 to S
66
Many proteins of the \_\_\_\_\_, when absent or defective, are associated with human ciliary diseases (ciliopathies).
transition zone
67
Basal bodies are typically ______ in diameter and _____ in length.
150-200 nm; 500 nm
68
Axonemes also provide \_\_\_\_\_\_.
the tracks for movement within cilia
69
Basal bodies are the core anchors from which ____ are formed.
cilia
70
\_\_\_\_\_\_\_\_ are derived from centrioles.
Basal bodies
71
\_\_\_\_\_ occurs during the G1 to S-phase
Centriole duplication

M2M Unit IV (11 decks)

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