Extracellular matrix Flashcards

1
Q

What are the basic elements of the ECM?

A

1) Ground substance2) Fibers3) 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 fibers4) Viscous5) Acts as lubricant, barrier, and provides structure6) 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) Glycosaminoglycans2) Proteoglycans3) Multiadhesive glycoproteins4) 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 adhesion2) Proliferation3) Differentiation4) Glomerular filtration5) Development6) 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 integrins2) One for collagen fibers3) One for proteoglycans

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22
Q

Where is fibronectin found?

A

1) Connective tissue2) Blood plasma3) 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
Q

Where is tenascin found?

A

Embryonic tissue

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26
Q

Where is chondronectin found?

A

Cartilage

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27
Q

Where is osteonectin found?

A

Bone

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28
Q

Collagen is primarily made up of what amino acids?

A

1) Glycine2) Hydroxyproline3) Hydroxylysine

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29
Q

What is the most abundant collagen type?

A

Type I

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30
Q

What is the tissue distribution of type I collagen?

A

1) Dermis2) Tendon3) Bone4) Dentin5) Cementum6) Fibrocartilage7) Organ capsules

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31
Q

What is the function of type I collagen?

A

Resistance to tension

32
Q

What is the tissue distribution of type II collagen?

A

Hyaline and elastic cartilage

33
Q

What is the function of type II collagen?

A

Resistance to pressure

34
Q

What is the tissue distribution of type III collagen?

A

1) Spleen2) Liver3) Lymph nodes4) Smooth muscle5) Skin6) Lungs

35
Q

What is the function of type III collagen?

A

Maintains structure in expandable organs

36
Q

What is the tissue distribution of type IV collagen?

A

Basal lamina

37
Q

What is the function of type IV collagen?

A

Support of delicate structures and filtration

38
Q

What is the tissue distribution of type V collagen?

A

Dermis

39
Q

What is the function of type VII collagen?

A

Anchors the basal lamina to the lamina reticularis

40
Q

Reticular fibers are composed of what type of collagen?

A

Type III

41
Q

What stains could be used to detect reticular fibers?

A

1) Silver Methods2) PAS Reaction

42
Q

What are the attributes of elastic fibers?

A

1) Slender and branched (loose CT)2) Densely arranged in bundles (ligamentum flavum)3) Detected by resorcin-fuschin or Verhoeff

43
Q

What stains detect elastic fibers?

A

1) Resorcin-fuschin2) Verhoeff

44
Q

How is nonfibrous elastic tissue arranged?

A

Fenestrated sheets or lamellae

45
Q

Which cells synthesize elastic fibers?

A

1) Fibroblasts2) Smooth muscle cells3) Chondroblasts

46
Q

Which three components must be synthesized to form elastic fibers?

A

1) Proelastin2) Microfibril-associated glycoprotein3) Fibrillin (1 and 2)

47
Q

What is the defect associated with EDS IV?

A

Mutation in COL3A1 gene encoding type III collagen (reticular fibers)

48
Q

What are the clinical consequences of EDS IV?

A

1) Varicose veins2) Aortic rupture3) Intestinal rupture

49
Q

What is the defect associated with EDS VI?

A

Defective hydroxylation of lysine - destabilizes collagen strength

50
Q

What are the clinical consequences of EDS VI?

A

1) Hyperelasticity of the skin2) Rupture of the eyeball

51
Q

What is the defect associated with EDS VII?

A

Mutations of the COL1A1 and COL1A2 genes encoding type I collagen

52
Q

What are the clinical consequences of EDS VII?

A

1) Joint dislocation2) Hypermobility of joints

53
Q

What is the defect associated with scurvy?

A

Tropocollagen molecules cannot aggregate into fibrils due to decreased hydroxylation of proline caused by vitamin C deficiency

54
Q

What are the clinical consequences of scurvy?

A

1) Gum ulceration2) Hemorrhages

55
Q

What is the defect associated with osteogenesis imperfecta?

A

Mutations in the COL1A1 gene leading to reduction in type I collagen production

56
Q

What are the clinical consequences of osteogenesis imeprfecta?

A

1) Spontaneous fractures2) Cardiac insufficiency

57
Q

What is the defect associated with Strickler syndrome?

A

Mutation in COL2A1 gene encoding for type II collagen

58
Q

What are the clinical consequences of Strickler syndrome?

A

1) Myopia2) Hypoplasia of mandible3) Arthritis

59
Q

What is the defect associated with Marfan syndrome?

A

Mutation of fibrillin 1 gene on chromosome 15

60
Q

What are the clinical consequences of Marfan syndrome?

A

1) Aortic aneurysm or rupture2) Myopia3) Detached lens4) Skeletal defects5) Pectus excavatum6) Scoliosis

61
Q

What makes up the basal lamina?

A

Lamina lucida (variable) + lamina densa

62
Q

What part of the basal lamina is secreted by epithelial, muscle, adipose, and Schwann cells?

A

Lamina densa

63
Q

What makes up the basal membrane?

A

Basal lamina + lamina fibroreticularis

64
Q

What part of the basal membrane is secreted by fibroblasts?

A

Lamina fibroreticularis

65
Q

What makes up the lamina lucida?

A

1) Laminin2) Entactin (nidogen)3) Integrins4) Dystroglycans

66
Q

What are the transmembrane receptors for laminin?

A

1) Integrins2) Dystroglycans

67
Q

What is the lamina densa composed of?

A

1) Type IV collagen2) Fibronectin3) Perlecan

68
Q

Type IV collagen, fibronectin, and perlecan make up what layer of the basal membrane?

A

Lamina densa

69
Q

What is the lamina fibroreticularis composed of?

A

1) Fibronectin 2) Type I collagen3) Type III collagen

70
Q

How is the basal lamina anchored to lamina fibroreticularis?

A

Anchoring fibrils of :1) Type VII collagen2) Fibrillin3) FIbronectin

71
Q

The external lamina surrounds what elements?

A

Nonepithelial cells (muscle, adipocytes, Schwann cells)

72
Q

Nonepithelial cells are surrounded by what type of basal lamina?

A

External lamina

73
Q

What are the functions of the basement membrane?

A

1) Adhesion interface between epithelium and ECM2) Molecular filter3) Regulation of cell differentiation, proliferation, and organization4) Regeneration of cells

74
Q

In Goodpasture syndrome, antibodies are formed against what type of collagen?

A

Type IV collagen

75
Q

Antibodies against type IV collagen are formed in what disease?

A

Goodpasture syndrome