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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
Q

2nd features of collagen

A

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
Q

Collagen produced from

A

Fibroblasts

27
Q

Collagen biosyntehsis

A

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
Q

Hyp stabilizes

A

Interactions between fibrils

29
Q

Scurvy cause

A

Vit C deficiency that affects Hyp and Hly synthesis (prolylhydroxylase needs vitamin C as cofactor)

30
Q

Scurvy symptoms

A 
Fatigue/depression
Dry hair, mouth, eyes, skin 
Easy bruising, infection, nosebleeds, inflamed gums 
Swollen joints 
Vasomotor instability
31
Q

Types of collagne fibers

A 
Fibrillar - 1,2,3,5,11
Beaded - 6
Network - 4, 8
Fibril-associated - 9,12,14 
Anchoring - 7
32
Q

OI inheritnace

A

Auto dom

33
Q

OI cause

A

Substitution of different amino acids for glycine in collegen 1 fibril

34
Q

OI characteristics

A

Bone fractures, poor spine curvature, loose joints, poor muscle tone
DI

35
Q

Ethlers-Danlos syndrome inheritance

A

Some are haploinsufficient (reduce amount made)

Some are auto-dom (gain of toxic function)

36
Q

Ethlers-Danlos cause and characteristics

A

Easily damaged, burised, and stretchy skin

Defect in collagne type 3

37
Q

EGF pathway

A

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
Q

Dystrophic Epidermolysis Bullosa inheritance

A

Auto recessive

39
Q

DEB characteristics and cause

A

Collegne type 7

Blistering, trauma to skin leading to separation of dermis and epidermis

40
Q

In normal cells, keratinocytes

A

Synthesize type 7 collagen molecules which assemble into anchoring fibrils

41
Q

In DEB cells

A

Only a few anchoring fibrils

42
Q

Tx of DEB

A

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
Q

GAGs - purpose, structure, and mech

A

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
Q

GAGs participate in signaling by

A

controlling diffusion rates

45
Q

2 GAGs

A

Glucosamine, glucuronate

46
Q

Proteoglycans example

A

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
Q

Proteoglycans unique in that

A

Percentage of carbs is way higher than proteins

48
Q

Core protein of proteoglycan is

A

Made in the ER, glycosylated, sulfated, and secreted into the ECM

49
Q

Structure of proteoglycan

A

Protein, link tetrasaccharide, GAG

50
Q

Proteoglycan lysosomal disorder

A

Mucopolysaccharidoses

Affect mostly bone and cartilage but also some mental retardation

51
Q

Metalloproteases

A

Cleavage of ECM components
All are metalloproteases (matrix metalloproteases, adamalysins, and meprins)

Histidine chelates with zinc ions
Can be anchored or secreted

52
Q

Branching of submandibular glands

A

MMP2 and MMP14 are involved at the end bud tip while MMP3 is involved in side branching

53
Q

HSPGs

A

Heparan sulphate proteoglycans

Bind FGFs and create concentration gradient to control cell fate

54
Q

FGF10 and FGF7

A

FGF 10 - promotes elongation

FGF 7 - promotes branching (lower affinity and diffuses broadly)

55
Q

FGF binding

A

Binds perlecan and requires heparanse in order to cleave so it can bind to receptor

56
Q

Osteoarthritis mechanism

A

Either overactivation of MMPs or osteoclasts

57
Q

Inflammation and fibrosis mechanism

A

Injury/infl signals fibroblasts and myofibroblasts to secrete TGF-B…means increase in ECM synthesis and decreases in MMP expression and ECM destruction

58
Q

Post-myocardial infarction

A

Inject ECM scaffolds derived from decellularized myocardial tissue

59
Q

Perlecan has been shown

A

To be neuroprotective after acute stroke by promoting the secretion of VEGF and fostering angiogenesis

60
Q

Repair of chronic wounds and bone defects

A

Increased growth factor affinity for the ECM via domain from placental growth factor 2 (PIGF2)

Foundations test 3 (15 decks)

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