Cilia and ciliopathies

Question 1

Basal bodies

Answer 1

core anchors from which cilia are formed

• MT-rich cylinder formed from 9 triplet MTs (A-B-C)
• 150-200 nm in diameter and 500 nm in length
• polarized
• formed beginning at prox end and distal end nucleates the cilium

Question 2

axoneme

Answer 2

• structural skeleton
• provide tracks for movement within cilia
• doublet MTs (A-B)
• polar
• (+) ends resides at the ciliary tip
• huge length range

Question 3

transition zone

Answer 3

• links the basal body to the axoneme and to the ciliary membrane
• “gatekeeper” = limits diffusion of membrane and soluble proteins into and out of the cilium
• make sure ciliary membrane is a distinct compartment for cellular signaling

Question 4

ciliary membrane

Answer 4

continuous with the cellular PM

• compartmentalized by the transition zone
• has unique phospholipids and receptor molecules

Question 5

cargo is transported along the axoneme by ______, which is ___directional with _______ motors and _______ protein complex directing movement to the ciliary tip.

Answer 5

• intraflagellar transport (IFT)
• bi
• kinesin
• IFT-B

Question 6

retrograde transport of the axonemes occurs by cytoplasmic _______ motor driven transprort with the _______ protein complex

Answer 6

• dynein 2

- IFT-A

Question 7

process of ciliogenesis

Answer 7

centrioles/basal bodies are assembled, followed by the formation of the cilium

Question 8

basal bodies are derived from

Answer 8

centrioles

Question 9

centrioles interchange between their function at centrosomes to

Answer 9

organize the cellular array of cytoplasmic MTs during interphase and mitosis

Question 10

upon ciliogenesis, the older of the two centrioles functions as

Answer 10

the basal body or anchor

Question 11

centriole duplication occurs during

Answer 11

G1-S phase cell cycle boundary coincident with DNA synthesis (like DNA synth, many same molecular components used and is tightly regulated)

Question 12

ciliogeneiss normally occurs during ______ of the cell cycle by assembling from the _______ of the centriole pair.

Answer 12

• G1 or G0

- mother centriole

Question 13

ciliogenesis begins when

Answer 13

the distal end of the basal body is capped by a “ciliary vesicle”

Question 14

after distal end is capped by a “ciliary vesicle,”

Answer 14

MT doublets assemble into the ciliary vesicle before the entire structure fuses with the plasma membrane of the cell

Question 15

motile cilia are required for

Answer 15

the movement of fluid in the respiratory, neural and reproductive tracts

Question 16

motility of cilia is produced by

Answer 16

axonemal dynein dependent sliding motion between the double MTs of the ciliary axoneme

Question 17

motile cilia typically have a _______ MT arrangement

Answer 17

9+2 = 9 doublet MTs organized around a central pair of singlet MTs

Question 18

non-motile, sensory or primary cilia posses a ______ MT arrangement and lack _______

Answer 18

• 9+0

- axonemal dynein arms