Lecture 6 Flashcards

1
Q

Intermediate filament subunits differ from Microtubules and Microfilaments in that they are ____ rather than globular.

A

FIBROUS

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2
Q

All 5 types of IFs have the same alpha-helical region consisting of about 310 AAs, but the subunits differ in the size and sequence of their ____ and ____ termini.

A

AMINO and CARBOXY termini

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3
Q

IFs are long, unbranched structures about ___nm in diameter.

A

10nm

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4
Q

IF monomer subunits pair to form ____, which line up side by side (staggered) to form _____, which bind end to end to form _____. The final filament is composed of 8 tetramers to form a rope-like filament, which is stronger than MFs and MTs.

A

Monomers pair to form DIMERS, which line up side by side (staggered) to form TETRAMERS, which bind end to end to form PROTOFILAMENTS. The final filament is composed of 8 tetramers

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5
Q

Type I and II IFs are ____-keratins and ____- keratins respectively, and are predominant types occurring in various _____ and _____-derived cells.

A

ACID and BASE-keratins respectively, and are predominant types in various EPITHELIAL and EPITHELIAL-derived cells.

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6
Q

There are 5 subclasses of Type III IFs, one of which is Peripherin, found in neurons of _____ nervous system.

A

Found in neurons of PERIPHERAL nervous system.

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7
Q

Type IV IFs are ____filament proteins

A

NEUROfilament

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8
Q

Type V IFs are nuclear _____, and are present in all cells.

A

Nuclear LAMINS

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9
Q

IF associated proteins (IFAPs) cross-link IFs with one another and also with ____ and ____.

A

MFs and MTs

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10
Q

The main IFAP is ____.

A

PLECTIN

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11
Q

All cells have nuclear lamin (Type ___ IF), but will have different cytoplasmic IF types depending on the cell type.

A

All have nuclear lamin (Type V), but will have different cytoplasmic IF types depending on the cell type.

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12
Q

IFs are important in providing mechanical support , help maintain cell adhesion via _____ associated with desmosomes, aid in ____ repair (keratin), and stabilize _____ (desmin.)

A

Help maintain cell adhesion via KERATIN associated with desmosomes, aid in WOUND repair (keratin), and stabilize SARCOMERES (desmin.)

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13
Q

Nuclear lamins are located along the inner nuclear _____ and less so in the nucleoplasm.

A

Inner nuclear MEMBRANE

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14
Q

There are 5 key proteins associated with the Nucleoskeleton (LAMSE):

  1. ____ A and B
  2. ____
  3. ____
  4. ____
  5. ____
A
  1. LAMINS A and B
  2. ACTIN
  3. MYOSIN
  4. SPECTRIN
  5. EMERIN
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15
Q

Lamins mainly contribute to stability and _____ of nuclear envelope, but are also involved in chromatin organization, transcription, replication, differentiation, and signaling.

A

Mainly contribute to stability and STIFFNESS

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16
Q

Actin plays a role in establishing and maintaining nuclear _____, and it can be found in 3 forms in the nucleus:

  1. _____
  2. _____ filaments that interact with nuclear lamina and other members of nucleoskeleton
  3. _____
A

Establishing nuclear STRUCTURE

  1. MONOMERIC
  2. SHORT filaments that interact with nuclear lamina and other members of the nucleoskeleton
  3. OLIGOMERIC
17
Q

The motor protein _____ is also present in the nucleus, where it is concentrated near the membrane (though it can be found in the nucleoplasm as well.) It associates at the inner nuclear membrane with lamin, actin, and ____.

A

Motor protein MYOSIN. It associates with lamin, actin, and EMERIN.

18
Q

Alpha-spectrin is present in the nucleus of non-_____ cells, and confers _____ to the nucleus. It may also be involved in the repair of interstrand DNA cross-links

A

Present in the nucleus of non-ERYHTROID cells, and confers ELASTICITY to the nucleus.

19
Q

Emerin is a protein embedded in the inner nuclear membrane and directly binds _____ to anchor the nuclear _____ to the nuclear envelope.

A

Directly binds LAMINS to anchor the nuclear LAMINA to the nuclear envelope.

20
Q

LINC complex, found in the nuclear envelope, is responsible for mechanically linking the _____ with the _____.

A

Mechanically linking the CYTOSKELETON to the NUCLEOSKELETON

21
Q

Interactions of cells at cell junctions activates signalling pathways that transmit ____ forces to the cytoskeleton –> the cytoskeleton attaches to ____ complexes on the nuclear envelope which transmit the mechanical force to the nucleoskeleton.

A

Activates signalling pathways that transmit MECHANICAL forces to the cytoskeleton –> the cytoskeleton attaches to LINC complexes at the nuclear envelope which transmit the mechanical force to the nucleoskeleton.

22
Q

LINC complexes consist of ____ proteins (typically Nesprin) in the outer nuclear membrane and ____ proteins in the inner nuclear membrane. These proteins interact with each other within the perinuclear space.

A

LINC complexes consist of KASH proteins (typically Nesprin) in the outer nuclear membrane and SUN proteins in the inner nuclear membrane. These proteins interact with each other in the perinuclear space.

23
Q

SUN proteins help anchor the LINC complex by binding the nuclear _____. They are also important in keeping the inner and outer nuclear membranes together.

24
Q

Nesprins bind ____ in the cytoplasm. Different Nesprins on LINC complexes bind different cytoskeletal filaments, which confers functional ____ to the linking process.

A

IFs. Different Nesprins bind different cytoskeletal filaments, which confers functional DIVERSITY to the linking process

25
HSV 1 causes hyper-_____ and rearrangemnet of Emerin in the nuclear envelope. This causes Emerin to dissociate from the nuclear lamina and disrupts the shape/integrity of the nuclear membrane.
Hyper-PHOSPHORYLATION of Emerin
26
Epidremolysis Bullosa Simplex is caused by genetic mutations in ____. This causes the basal layer of the epidermis to be susceptible to mechanical stress/friction which leads to _____ formation due to cytolysis.
Mutations in KERATIN. Epidermis is susceptible to mechanical stress/friction which leads to BLISTER formation.
27
Emery-Dreifuss Muscular Dystrophy is a type of Laminopathy and has two major forms: 1. Autosomal _____ 2. X-linked
Autosomal DOMINANT
28
EDMD symptoms usually appear in first ____ of life and include cardiac disease, cadio_____, progressive muscle wasting especially in upper arms and lower legs, and early _____ of major tendons in elbows and neck.
Usually appear in first DECADE of life and include cardiac disease, cardioMYPATHY, progressive muscle wasting, and early CONTRACTURES in major tendons of elbows and neck.
29
Autosomal dominant EDMD is caused by mutations in ____A/C which compromises the integrity of the nuclear envelope.
LAMINA
30
X-linked EDMD is caused by mutations in _____, which compromises the integrity of nuclear envelope and causes _____ to mislocalize.
EMERIN, which compromises the integrity of nuclear envelope and causes NESPRIN to mislocalize.
31
Hutchinson-Gilford Progeria is caused by an autosomal-_____ mutation in _____A gene, which causes it to lose 50 AAs, forming _____ instead.
Autosomal-DOMINANT mutation in LAMIN-A gene, which causes it to lose 50 AAs, forming PROGERIN.
32
The nucleus of cells in Hutchinson-Gilford patients show abnormal shape and _____ that increases with age.
Abnormal shape and BLEBBING
33
Clinical symptoms of HGPS include premature ____, sever growth _____, loss of subQ ____, hair loss, progressive atherosclerosis and cardiovascular disease
Include premature AGING, sever growth RETARDATION, loss of subQ FAT, hair loss, progressive atherosclerosis, and cardiovascular disease.
34
Epithelial cancers are characterized by upregulation of the IF cross-linking protein _____. This protein has a specificity for premalignant _____ ductal adenocarcinoma cells, and localizes to the cell membrane in _____ cancer cells. It may be used as a bio-imaging maker for detection of this cancer.
Upregulation of PLECTIN. It has a speceficity for premalignant PANCREATIC ductal adenocarcinoma cells, and localizes to the cell membrane in PANCREATIC cancer cells.