introduction Flashcards
exam 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
Progeria
a versatile, self-assembling organelle
nucleus
______ ______ form a tough network beneath the nuclear membrane and connect the nuclear membrane to chromatin
nuclear Lamins
example of laminopathies
progeria, and restrictive dermopathy
24, ______, defects cause nuclear disorganization and identify ___________ asa lethal neonatal laminopathy
24, Face-1, defect causes restrictive dermopathy
Lamins, a type of ________ filament protein, are responsible for connecting ______ to the inner ______ _____
Lamins, a type of intermediate filament protein, are responsible for connecting chromatin to the inner nuclear membrane
During i______, when the nuclear envelope is intact, lamins are in the _________ state
During interpahse, when the nuclear envelope is intact lamins are in the dephosphorylated state
Early in mitosis, Lamins are phosphorylates by a _____, causing the _______ ______ connection to break, thus beginning the process of _____ _____ disassembly
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
Late in mitosis, a ________ returns lamins to the _________ state, thus permitting nuclear membrane _____ to occur
Late in mitosis, a phosphatase returns lamins to the dephosphorylated state, thus permitting nuclear membrane assembly to occur
CAG repeats
Huntington’s
nuclear transport occurs via _____ ___
nuclear pores
nuclear localization signal for
import
required for cargo import
NLS
____ ______ bind to NLS on cargo and escort cargo to nuclear pore
Chaperone proteins
cargo smaller than 5-10 kDa can enter nucleus via ______ through nuclear pores, but larger cargo must be _______ transported through nuclear pores
smaller cargo enter through diffusion and larger must be transported actively
specializes in exporting tumor suppressors, apoptosis inducers , and anti-proliferative molecules that exert their normal biological activities via binding to DNA
exportin-1
cancer cells greatly ____-____ exportin-1
over-express
tru or false: over expressing of exportin-1 results in a dearth of tumor supressors leading to excessive cell proliferation and tumor growth
true
inhibitors of nuclear export
selective inhibitors of nuclear export (SINEs)
analogous to nuclear import, _______ and ____-_____ complexes destined for export have NLS that are recognized by _______
proteins and ribonucleo-protein are recognized by exportins
exportins recognize a specific _________ on the nuclear pore and bind it
5-Gpp site
often the key to revealing fundamentally important normal and disease mechanisms/pathways
Rare diseases
altered nuclear import disease
Huntington’s
the ______ ____ Huntingtin is mutated via addition of ________ residues and the mutant protein ends up in the ______
the cytoplasmic protein Huntingtin is mutated via addition of glutamine residues and the mutant protein ends up in the nucleus
