introduction

Question 1

a genetic disease in which patients age so rapidly they die in their second decade of life from advanced atherosclerosis, which is typically a disease of the elderly

Answer 1

Progeria

Question 2

a versatile, self-assembling organelle

Answer 2

nucleus

Question 3

______ ______ form a tough network beneath the nuclear membrane and connect the nuclear membrane to chromatin

Answer 3

nuclear Lamins

Question 4

example of laminopathies

Answer 4

progeria, and restrictive dermopathy

Question 5

24, ______, defects cause nuclear disorganization and identify ___________ asa lethal neonatal laminopathy

Answer 5

24, Face-1, defect causes restrictive dermopathy

Question 6

Lamins, a type of ________ filament protein, are responsible for connecting ______ to the inner ______ _____

Answer 6

Lamins, a type of intermediate filament protein, are responsible for connecting chromatin to the inner nuclear membrane

Question 7

During i______, when the nuclear envelope is intact, lamins are in the _________ state

Answer 7

During interpahse, when the nuclear envelope is intact lamins are in the dephosphorylated state

Question 8

Early in mitosis, Lamins are phosphorylates by a _____, causing the _______ ______ connection to break, thus beginning the process of _____ _____ disassembly

Answer 8

early in mitosis, lamins are phosphorylated by a kinase, causing the chrmatin-nuclear membrane connection to break, thus beginning the process of nuclear membrane disassembly

Question 9

Late in mitosis, a ________ returns lamins to the _________ state, thus permitting nuclear membrane _____ to occur

Answer 9

Late in mitosis, a phosphatase returns lamins to the dephosphorylated state, thus permitting nuclear membrane assembly to occur

Question 10

CAG repeats

Answer 10

Huntington’s

Question 11

nuclear transport occurs via _____ ___

Answer 11

nuclear pores

Question 12

nuclear localization signal for

Answer 12

import

Question 13

required for cargo import

Answer 13

NLS

Question 14

____ ______ bind to NLS on cargo and escort cargo to nuclear pore

Answer 14

Chaperone proteins

Question 15

cargo smaller than 5-10 kDa can enter nucleus via ______ through nuclear pores, but larger cargo must be _______ transported through nuclear pores

Answer 15

smaller cargo enter through diffusion and larger must be transported actively

Question 16

specializes in exporting tumor suppressors, apoptosis inducers , and anti-proliferative molecules that exert their normal biological activities via binding to DNA

Answer 16

exportin-1

Question 17

cancer cells greatly ____-____ exportin-1

Answer 17

over-express

Question 18

tru or false: over expressing of exportin-1 results in a dearth of tumor supressors leading to excessive cell proliferation and tumor growth

Answer 18

true

Question 19

inhibitors of nuclear export

Answer 19

selective inhibitors of nuclear export (SINEs)

Question 20

analogous to nuclear import, _______ and ____-_____ complexes destined for export have NLS that are recognized by _______

Answer 20

proteins and ribonucleo-protein are recognized by exportins

Question 21

exportins recognize a specific _________ on the nuclear pore and bind it

Answer 21

5-Gpp site

Question 22

often the key to revealing fundamentally important normal and disease mechanisms/pathways

Answer 22

Rare diseases

Question 23

altered nuclear import disease

Answer 23

Huntington’s

Question 24

the ______ ____ Huntingtin is mutated via addition of ________ residues and the mutant protein ends up in the ______

Answer 24

the cytoplasmic protein Huntingtin is mutated via addition of glutamine residues and the mutant protein ends up in the nucleus

Question 25

Huntingtin is susceptible to _______ and that it is the smaller peptides that result in that are able to _____ through the nuclear pores

Answer 25

proteolysis and able to diffuse