ECM and adhesion Flashcards

1
Q

what is a complex network of secreted macromolecules

A

ECM

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2
Q

what does GAG stand for

A

glucosaminoglycans

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3
Q

the ECM provides a 3D framework for

A

organisation of tissues and basic cellular processes

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4
Q

the ECM regulates things

A

proliferation, differentiation, migration, cell-cell interaction

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5
Q

composition of ECM macromolecular network

A

collagens, elastin, glycoproteins, proteoglycans

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6
Q

example of ECM disease- too much ECM

A

fibrosis

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7
Q

how much of total protein in body is collagen

A

25%

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8
Q

what are the primary structural components in connective tissue

A

collagens

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9
Q

how many different types of collagens have been identified

A

> 25

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10
Q

what is the structure of collagen and amino acid sequence

A

triple helical structure Gly-X-Y ie. every 3rd amino acid is glycine

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11
Q

2 main categories of collagen

A

fibrillar (fibril forming, well organised banded fibrils) and non-fibrillary (form microfibrils/ mesh like structures)

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12
Q

type I and II are what type of collagen

A

fibrillar

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13
Q

type IV and VI are what type of collagen

A

non-fibrillary

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14
Q

types I and II are major components in ? and provide ?

A

tendons, ligaments, skin- provide high tensile strength

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15
Q

type IV and VI are structural components of ? and provide ?

A

basement membranes- provide anchorage of cells and filtration

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16
Q

what is the name for brittle bone disorder

A

osteogenesis imperfecta

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17
Q

osteogenesis is caused by genetic defects in which type of collagen

A

collagen I

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18
Q

what are 5 symptoms of OI

A

fragile bones, think skin, abnormal teeth, think sclera, weak tendons

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19
Q

what amino acid is mutated in OI

A

glycine to a more bulky one

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20
Q

OI is what type of mutation

A

single base

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21
Q

which type of OI is most common I or II

A

type I (50%)

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22
Q

Type I OI is autosomal dominant/ recessive

A

dominant

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23
Q

which type of OI is the mildest

A

Type I

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24
Q

which genes are mutated in OI type I

A

COL1A1 and A2 genes on chromosomes 7 and 17

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25
Q

which type of OI is more severe

A

type II

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26
Q

what type of mutation is type II usually and is it dominant/reccessive

A

de novo, dominant

27
Q

why do type II OI babies not often survive

A

bones so fragile

28
Q

what is the predominant protein in elastic fibres

A

elastin

29
Q

elastin gives flexibility to what 4 things

A

blood vessels, lungs, ligaments, skin

30
Q

can elastin stretch in 2D or 3D

A

2D (1000x more stretchy than collagen)

31
Q

what 2 amino acids is elastin rich in

A

glycine and proline (like collagen)

32
Q

what amino acid makes elastin different to collagen and why

A

valine interacts with hydrophobic domains which gives it elasticity

33
Q

elastin fibres are covered by what and why

A

glycoprotein fibrillin- so stretchy so needs control/ stretch without breaking

34
Q

what syndrome is from mutations in fibrillin gene

A

marfan syndrome eg. Abraham lincoln

35
Q

what are some characteristics of Marfan syndrome

A

tall stature, long arms and legs, spider fingers (arachnodactyl), loose joints, floppy cardiac valves, eye problems, aortic aneurysms

36
Q

what helps to anchor elastin fibres to other ECM proteins

A

fibrillin- structural role

37
Q

fibrillin controls amount of what growth factor

A

TGF-b

38
Q

in marfan syndrome TGF-b is not bound with ECM which causes what

A

accumulation in lungs, heart and other tissues which changes ECM in these organs especially where there is a lot of elastic fibres

39
Q

what are the 2 sugar containing proteins in animal cells

A

glycoproteins and proteoglycans

40
Q

what is a glycoprotein

A

polypeptide chain with oliogosaccharide/ carbohydrate stuck on end

41
Q

glycoproteins play a role in what immunity and why

A

innate immunity- receptors on cell surface of bacteria, viruses, toxins

42
Q

2 examples of glycoproteins

A

laminin, fibronectin

43
Q

3 roles of laminin

A

cell adhesion, cell migration, cytoskeletal organisation (connects epithelial cells to connective tissue)

44
Q

5 roles of fibronectin

A

cell: adhesion, migration, shape, differentiation, cytockeltal organisation

45
Q

what is composed of a peptide chain with covalently bound sugars (proteins conjugated to polysaccharides with serial repeat units)

A

proteoglycan

46
Q

do glycoproteins or proteoglycans have more carbohydrates

A

proteoglycans

47
Q

what is the carb and protein % in proteoglycans

A

95%, 5%

48
Q

proteoglycans: core protein and what?

A

GAGs (mucopolysaccharide)

49
Q

proteoglycan side chains (GAGs) contain ? group with ? charge

A

sulphate, -ve

50
Q

are proteoglycans water attracting

A

yes

51
Q

proteoglycans are ? forming

A

gel

52
Q

proteoglycans can form large ? in most tissues

A

aggregates

53
Q

proteoglycan aggregates give what to tissues

A

compressive strength

54
Q

proteoglycans in tissues increase/ decrease with age

A

decrease

55
Q

what happens to proteoglycans with age

A

shorter and fewer side chains, draw and hold less water, more likely to be damaged (dry and brittle)

56
Q

proteoglycans are important in aging and ?

A

healing

57
Q

how are proteoglycans important in healing

A

upregulated in damaged area, cause initial swelling (oedema), more nutrients and GF

58
Q

cell adhesion :

A

process by which cells interact and attach to neighbouring cells using cell surface molecules

59
Q

direct contact cell adhesion is between

A

cell surfaces

60
Q

indirect interactions cells attach to

A

surrounding ECM

61
Q

cell adhesion is involved in 3 things

A

signal transduction for cells to detect and respond to changes in surroundings, regulate cell migration, regulate tissue development in multicellular organisms

62
Q

cell adhesion molecules CAMs belong to 5 protein families

A

cadherins, Ig superfamily, mucins, selectins, integrins

63
Q

CAMs have homophilic (2 ) and heterophilic interactions (3)

A

cadherins, Ig super family

mucins, selectins, integrins