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Foundations test 3 > ECM, Collagen and Disease > Flashcards

ECM, Collagen and Disease Flashcards

1
Q

Interstitial matrix is made of

A

Polysaccharides and fibrous proteins

Acts as shock absorber

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2
Q

Basal lamina

A

Divides cell from ECM

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3
Q

Most abundant ECM molecules

A

Collagen and fibronectin

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4
Q

Fibronectins play role in

A

Wound healing, phagocytosis, and platelet-platelet cohesion

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5
Q

Soluble fibronectins

A

Clotting

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6
Q

insoluble fibronectins

A

Connects ECM to intracellular actin via integrins

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7
Q

Fibronectin helps give what kind of strenght?

A

Tensile

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8
Q

Mechanism of fibroectin

A

Mechanical tension regulates unfolding and exposes cryptic binding sites

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9
Q

Focal adhesion

A

Complex that connects fibronectin in the ECM to actin filaments inside the cell using integrins

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10
Q

Integrins require

A

Ions

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11
Q

Hemidesmosome vs. desmosome

A

Hemi - involves connections to keratin filaments inside the cell (between ECM and cell)
Desm - cell-cell adhesion

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12
Q

Glanzmann’s dz mutation and symptoms

A

Fibrinogen mutation…bleeding gums and nose bleeds, no platelet aggregation…mild osteoporosis

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13
Q

Laminin mutation effects

A

Severe blistering and other epithelial defects

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14
Q

LAD1 cause

A

Leukocyte adhesion deficiency 1
Failure to express a common B2 subunit of integrin that plays role in lymphocyte adhesion…prevents ability to migrate in and out of blood vessels

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15
Q

Gound substance componentns

A

Hyaluronan, proteoglycans, and glycoproteins

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16
Q

Proteoglycans

A

Core protein decorated with large GAGs

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17
Q

Integrins vital for

A

Cell adhesion to the basal lamina

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18
Q

Basal lamina important molecules

A 
Perlecan (protein)
Laminin
Type 4 collagen
Integrin
Nidogen
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19
Q

Laminin 1

A

Cross-shaped

Lamin self-assembles into a network that gives tensile strenght (stretching)

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20
Q

Collagen 4

A

Three superhelical strands in a cable that allow for knob like structures…lacks typical glycine every 3rd residue for tight helix of other collagnes

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21
Q

Alport syndrome cause

A

Defects in collagen 4 resulting in thickening of the glomerular kidney basal lamina

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22
Q

Alport syndrome symptoms

A

Glomerulonephritis, end-stage kidney dz, hematouria, and lenticonus (bulging of the eyes)

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23
Q

Collagen structure

A 
Tropocollagen subunit (2 intertwined pp chains rich in Pro and Gly with left handed alpha helical chains to form right handed supercoil)
Unusual amino acids (Hyp, Hyl, Aly)
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24
Q

Purpose of Hyp, Hyl, and Aly

A

Hyp - inferfibril and inferfiber H bonds
Hyl - undergoes glycosylation
Aly - deaminated lysine that provides covalent, interfibril cross-links

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25
2nd features of collagen
Left handed polyproline 2 helical conformation Prolines stabilize individual helix due to steric hindrance Glycine allows thick packing Prolines will be on outside and glycines on inside
26
Collagen produced from
Fibroblasts
27
Collagen biosyntehsis
Precursor alpha chain forms disulfide bonds to form procollagen...secreted...procollagen peptidase cleaves to tropocollagen....K oxidized to form allysine to forms bridges, H bonding (fibril)...Hyp mediated hydrogen bonding between fibrils (fiber)
28
Hyp stabilizes
Interactions between fibrils
29
Scurvy cause
Vit C deficiency that affects Hyp and Hly synthesis (prolylhydroxylase needs vitamin C as cofactor)
30
Scurvy symptoms
``` Fatigue/depression Dry hair, mouth, eyes, skin Easy bruising, infection, nosebleeds, inflamed gums Swollen joints Vasomotor instability ```
31
Types of collagne fibers
``` Fibrillar - 1,2,3,5,11 Beaded - 6 Network - 4, 8 Fibril-associated - 9,12,14 Anchoring - 7 ```
32
OI inheritnace
Auto dom
33
OI cause
Substitution of different amino acids for glycine in collegen 1 fibril
34
OI characteristics
Bone fractures, poor spine curvature, loose joints, poor muscle tone DI
35
Ethlers-Danlos syndrome inheritance
Some are haploinsufficient (reduce amount made) | Some are auto-dom (gain of toxic function)
36
Ethlers-Danlos cause and characteristics
Easily damaged, burised, and stretchy skin | Defect in collagne type 3
37
EGF pathway
EGF binds HER and causes dimemrization...tyrosine kinase cross phosphorylation...GRB2/SOS binds to phosphorylated tyrosines...Connect to RasGDP which is converted to RasGTP...this activates Ras and causes downstream cascade
38
Dystrophic Epidermolysis Bullosa inheritance
Auto recessive
39
DEB characteristics and cause
Collegne type 7 | Blistering, trauma to skin leading to separation of dermis and epidermis
40
In normal cells, keratinocytes
Synthesize type 7 collagen molecules which assemble into anchoring fibrils
41
In DEB cells
Only a few anchoring fibrils
42
Tx of DEB
Genetically engineered fibroblasts that elicit subclinical response that leads to one of EGF being produced by fibroblasts....leads to increase in type 7 collagen production
43
GAGs - purpose, structure, and mech
Resist compression forces Large amount of charge-charge repulsion Occupy signifcnace amount of volume relative to molar mass because of hydration/H bonding to water
44
GAGs participate in signaling by
controlling diffusion rates
45
2 GAGs
Glucosamine, glucuronate
46
Proteoglycans example
Hyaluronan or hyaluronate Unique among GAGs that it is synthesized in plasma membrane and secreted into the ECM...does not contain sulfate groups and not covalently attached to proetins
47
Proteoglycans unique in that
Percentage of carbs is way higher than proteins
48
Core protein of proteoglycan is
Made in the ER, glycosylated, sulfated, and secreted into the ECM
49
Structure of proteoglycan
Protein, link tetrasaccharide, GAG
50
Proteoglycan lysosomal disorder
Mucopolysaccharidoses | Affect mostly bone and cartilage but also some mental retardation
51
Metalloproteases
Cleavage of ECM components All are metalloproteases (matrix metalloproteases, adamalysins, and meprins) Histidine chelates with zinc ions Can be anchored or secreted
52
Branching of submandibular glands
MMP2 and MMP14 are involved at the end bud tip while MMP3 is involved in side branching
53
HSPGs
Heparan sulphate proteoglycans | Bind FGFs and create concentration gradient to control cell fate
54
FGF10 and FGF7
FGF 10 - promotes elongation | FGF 7 - promotes branching (lower affinity and diffuses broadly)
55
FGF binding
Binds perlecan and requires heparanse in order to cleave so it can bind to receptor
56
Osteoarthritis mechanism
Either overactivation of MMPs or osteoclasts
57
Inflammation and fibrosis mechanism
Injury/infl signals fibroblasts and myofibroblasts to secrete TGF-B...means increase in ECM synthesis and decreases in MMP expression and ECM destruction
58
Post-myocardial infarction
Inject ECM scaffolds derived from decellularized myocardial tissue
59
Perlecan has been shown
To be neuroprotective after acute stroke by promoting the secretion of VEGF and fostering angiogenesis
60
Repair of chronic wounds and bone defects
Increased growth factor affinity for the ECM via domain from placental growth factor 2 (PIGF2)

Foundations test 3 (15 decks)

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