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Extracellular matrix Flashcards

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1
Q

What are the basic elements of the ECM?

A

1) Ground substance
2) Fibers
3) Granulation tissue

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2
Q

What are the characteristics of the ground substance?

A

1) Amorphous intercellular material
2) Hydrated
3) Fills space between cells and fibers
4) Viscous
5) Acts as lubricant, barrier, and provides structure
6) Diffusion medium for metabolites, O2, CO2, growth factors

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3
Q

What are the molecular moieties of the ground substance?

A

1) Glycosaminoglycans
2) Proteoglycans
3) Multiadhesive glycoproteins
4) Water

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4
Q

What are glycosaminoglycans comprised of?

A

Linear polymers of disaccharides

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5
Q

How is the hydrating effect of glycoasminoglycans accomplished?

A

Negative carboxyl groups attract cations, which attracts extracellular fluid

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6
Q

In Grave’s disease, which extracellular matrix component is increased?

A

Glycosaminoglycans (e.g. hyaluronic acid, chondroitin sulfate)

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7
Q

What is another term for glycosaminoglycans?

A

Mucopolysaccharides

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8
Q

What are proteoglycans composed of?

A

Linear chains of glycosaminoglycans bound to a protein core

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9
Q

Linear chains of glycosaminoglycans bound to a protein core constitute what extracellular matrix component?

A

Proteoglycans

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10
Q

What is an aggrecan?

A

Proteoglycans non-covalently linked to hyaluronic acid

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11
Q

What is the term for proteoglycans non-covalently linked to hyaluronic acid?

A

Aggrecans

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12
Q

What is a syndecan?

A

Transmembrane proteoglycan

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13
Q

What type of proteoglycan serves as a co-receptor for fibroblast growth factor?

A

Syndecan

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14
Q

Syndecan serves as a co-receptor for what growth factor?

A

Fibroblast growth factor

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15
Q

What is the major proteoglycan found in the basement membrane and connective tissues?

A

Perlecan

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16
Q

What is the function of perlecan?

A

1) Cell adhesion
2) Proliferation
3) Differentiation
4) Glomerular filtration
5) Development
6) Growth factor binding

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17
Q

How does gene inactivation for perlecan affect skeletal development?

A

Impairs skeletal development

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18
Q

What is the importance of versican?

A

Cell-to-cell and cell-to-ECM interactions

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19
Q

What are multiadhesive glycoproteins?

A

Glycoproteins that molecularly tether the cell to extracellular elements

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20
Q

Which glycoproteins molecularly tether the cell to extracellular elements?

A

Multiadhesive glycoproteins

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21
Q

What are the three binding sites for multiadhesive glycoproteins?

A

1) One for integrins
2) One for collagen fibers
3) One for proteoglycans

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22
Q

Where is fibronectin found?

A

1) Connective tissue
2) Blood plasma
3) Embryonic tissue

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23
Q

Where is laminin found?

A

Basal lamina

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24
Q

Where is nidogen (enactin) found?

A

Basal lamina

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25
Where is tenascin found?
Embryonic tissue
26
Where is chondronectin found?
Cartilage
27
Where is osteonectin found?
Bone
28
Collagen is primarily made up of what amino acids?
1) Glycine 2) Hydroxyproline 3) Hydroxylysine
29
What is the most abundant collagen type?
Type I
30
What is the tissue distribution of type I collagen?
1) Dermis 2) Tendon 3) Bone 4) Dentin 5) Cementum 6) Fibrocartilage 7) Organ capsules
31
What is the function of type I collagen?
Resistance to tension
32
What is the tissue distribution of type II collagen?
Hyaline and elastic cartilage
33
What is the function of type II collagen?
Resistance to pressure
34
What is the tissue distribution of type III collagen?
1) Spleen 2) Liver 3) Lymph nodes 4) Smooth muscle 5) Skin 6) Lungs
35
What is the function of type III collagen?
Maintains structure in expandable organs
36
What is the tissue distribution of type IV collagen?
Basal lamina
37
What is the function of type IV collagen?
Support of delicate structures and filtration
38
What is the tissue distribution of type V collagen?
Dermis
39
What is the function of type VII collagen?
Anchors the basal lamina to the lamina reticularis
40
Reticular fibers are composed of what type of collagen?
Type III
41
What stains could be used to detect reticular fibers?
1) Silver Methods | 2) PAS Reaction
42
What are the attributes of elastic fibers?
1) Slender and branched (loose CT) 2) Densely arranged in bundles (ligamentum flavum) 3) Detected by resorcin-fuschin or Verhoeff
43
What stains detect elastic fibers?
1) Resorcin-fuschin | 2) Verhoeff
44
How is nonfibrous elastic tissue arranged?
Fenestrated sheets or lamellae
45
Which cells synthesize elastic fibers?
1) Fibroblasts 2) Smooth muscle cells 3) Chondroblasts
46
Which three components must be synthesized to form elastic fibers?
1) Proelastin 2) Microfibril-associated glycoprotein 3) Fibrillin (1 and 2)
47
What is the defect associated with EDS IV?
Mutation in COL3A1 gene encoding type III collagen (reticular fibers)
48
What are the clinical consequences of EDS IV?
1) Varicose veins 2) Aortic rupture 3) Intestinal rupture
49
What is the defect associated with EDS VI?
Defective hydroxylation of lysine - destabilizes collagen strength
50
What are the clinical consequences of EDS VI?
1) Hyperelasticity of the skin | 2) Rupture of the eyeball
51
What is the defect associated with EDS VII?
Mutations of the COL1A1 and COL1A2 genes encoding type I collagen
52
What are the clinical consequences of EDS VII?
1) Joint dislocation | 2) Hypermobility of joints
53
What is the defect associated with scurvy?
Tropocollagen molecules cannot aggregate into fibrils due to decreased hydroxylation of proline caused by vitamin C deficiency
54
What are the clinical consequences of scurvy?
1) Gum ulceration | 2) Hemorrhages
55
What is the defect associated with osteogenesis imperfecta?
Mutations in the COL1A1 gene leading to reduction in type I collagen production
56
What are the clinical consequences of osteogenesis imeprfecta?
1) Spontaneous fractures | 2) Cardiac insufficiency
57
What is the defect associated with Strickler syndrome?
Mutation in COL2A1 gene encoding for type II collagen
58
What are the clinical consequences of Strickler syndrome?
1) Myopia 2) Hypoplasia of mandible 3) Arthritis
59
What is the defect associated with Marfan syndrome?
Mutation of fibrillin 1 gene on chromosome 15
60
What are the clinical consequences of Marfan syndrome?
1) Aortic aneurysm or rupture 2) Myopia 3) Detached lens 4) Skeletal defects 5) Pectus excavatum 6) Scoliosis
61
What makes up the basal lamina?
Lamina lucida (variable) + lamina densa
62
What part of the basal lamina is secreted by epithelial, muscle, adipose, and Schwann cells?
Lamina densa
63
What makes up the basal membrane?
Basal lamina + lamina fibroreticularis
64
What part of the basal membrane is secreted by fibroblasts?
Lamina fibroreticularis
65
What makes up the lamina lucida?
1) Laminin 2) Entactin (nidogen) 3) Integrins 4) Dystroglycans
66
What are the transmembrane receptors for laminin?
1) Integrins | 2) Dystroglycans
67
What is the lamina densa composed of?
1) Type IV collagen 2) Fibronectin 3) Perlecan
68
Type IV collagen, fibronectin, and perlecan make up what layer of the basal membrane?
Lamina densa
69
What is the lamina fibroreticularis composed of?
1) Fibronectin 2) Type I collagen 3) Type III collagen
70
How is the basal lamina anchored to lamina fibroreticularis?
Anchoring fibrils of : 1) Type VII collagen 2) Fibrillin 3) FIbronectin
71
The external lamina surrounds what elements?
Nonepithelial cells (muscle, adipocytes, Schwann cells)
72
Nonepithelial cells are surrounded by what type of basal lamina?
External lamina
73
What are the functions of the basement membrane?
1) Adhesion interface between epithelium and ECM 2) Molecular filter 3) Regulation of cell differentiation, proliferation, and organization 4) Regeneration of cells
74
In Goodpasture syndrome, antibodies are formed against what type of collagen?
Type IV collagen
75
Antibodies against type IV collagen are formed in what disease?
Goodpasture syndrome

Cell Bio I (11 decks)

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