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Phase 1: weeks 1-5 > ECM > Flashcards

ECM Flashcards

1
Q

What is ECM

A

Acellular component of present in all tissues and organs

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2
Q

list the 2 main types of ECM

A

Interstitial connective tissue matrix

Basement membrane

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3
Q

What are the components of ECM

A

Fibres: elastin + collagen

Ground substance: Glycosaminoglycans + Proteoglycans + Glycoproteins

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4
Q

List places where ECM is found

A 
Bone
Cartilage
tendons 
blood vessel walls
subcutaneous fat
basemen membrane
dermal layer of skin 
cornea
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5
Q

Describe what collagen is and its structure

A

a large, insoluble, fibrous protein

made up of 3 collagen polypeptides which form a triple helix structure

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6
Q

What assembles collagen molecules into its strong helical structure?

A

presence of Gly(cine)-X-Y repeat motif

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7
Q

what are the two types of collagen, what cartilage are they present in and what functional property do they have

A

Fibrillar: found in type 1 and 2, provides strength. Found in skin, tendon, bone

Sheet/network forming: found in type 4, provide support and filter. Found in basement membrane.

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8
Q

Where is type 1 collagen found?

A

bone, tendon, skin (dermis), ligaments

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9
Q

Where is type 2 collagen found?

A

Hyaline cartilage

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10
Q

Where is type 3 collagen found?

A

granulation tissue, liver, bone marrow, lymphoid organs

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11
Q

Where is type 4 collagen found?

A

basement membrane

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12
Q

Where is type 5 collagen found?

A

linker to Basement membrane, in cornea

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13
Q

describe elastin, how is it arranged and what property does it have?

A

elastin is a structural protein arranged as fibres.

It is found in tissues which need stretch and recoil properties

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14
Q

What is needed to assemble elastin?

A

A structural glycoprotein called Fibrillin

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15
Q

What organs in elastin found in?

A

Lungs, skin, blood vessels, bladder

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16
Q

what is ground substance?

A

An amorphous (shapeless), colour, gelatinous material. It is found in the space between cells and fibres.

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17
Q

What forms a proteoglycan

A

A core protein and many GAGs

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18
Q

what does ground substance consist mainly of?

A

water

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19
Q

what is another name for GAGs

A

mucopolysaccharides

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20
Q

list the four different types of GAG and where they are found

A

hyaluronic acid - found in synovial fluid
keratAn sulphate - found in cartilage
HeparAn sulphate - found in basement membrane
chondroitin sulphate - found in cartilage

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21
Q

what properties do GAGs have?

A

They are hydrophilic so absorb water.
They provide cushioning and have hydrating properties.
They enabled ECM to withstand high compressive forces.

22
Q

what are proteoglycans also known as?

A

mucoproteins

23
Q

proteoglycans are mostly what?

A

carbohydrates (90-95%)

24
Q

list 4 types of Proteoglycans

A

Aggrecan
perlecan
Syndecan
Decorin

25
where is aggrecan found and what type of GAG is present?
In cartilage Chondrotiin sulphate + KERATAn sulphate
26
where is perlecan found and what type of GAG is present?
in basement membrane HepAran sulphate
27
where is syndecan found and what type of GAG is present?
in cartilage Chondroitin sulphate + heparan sulphate
28
where is decorin found and what type of GAG is present?
widespread in connective tissues Chondrotiin sulphate Dermatan sulphate
29
list 5 glycoproteins
``` fibrillin fibronectin entactin tenascin laminin ```
30
what is the role of fibronectin?
found in basement membrane attaches to integrins and creates a provisional matrix for keratinocytes to move along to close the wound
31
what makes the protein/fibres in the ECM?
Fibroblasts
32
what is collagen synthesised as, what post translational modification does it undergo and what is the protein assembly?
Procollagen Glycosylation + hydroxylation Triple helix
33
what is elastin synthesised as, what post translational modification does it undergo and what is the protein assembly?
tropoelastin hydroxylation fibrillin scaffold cross linked fibres
34
how are proteoglycans synthesised?
core protein is made on rER Addition of carbohydrates in Golgi apparatus delivered to extracellular component by exocytosis assembled with other ECM components by fibroblasts
35
If the ECM is never static what may it be undergoing?
Deposition Degradation Modification
36
how is ECM degraded when it is remodelled? wen may it need to be degraded for remodelling?
Broken down by proteases, MMPs, and elastase Used for wound repair, embryogenesis and angiogenesis
37
how do pathogens degrade ECM? give an example pathogen
pathogens secrete collagenase which enables them to invade the host. e.g. bacillus cereus
38
what are matrix metalloproteinases?
enzymes which break down proteins found in the ECM and need calcium and zinc to function
39
what is the basement membrane
a thin tough sheet of ECM
40
What are components of the busmen membrane ?
``` Collagen type 4 perlecan (PG) laminin (GP) entactin (gp) fibronectin (gp) ```
41
what are the 3 layers of the ECM?
Lamina lucida Lamina densa Lamina fibroreticularis
42
list some BM disorders
cancer epidermolysis bullosa goodpasture syndrome Diabetes mellitus
43
Describe goodpastures syndrome
autoantibodies fo collagen type 4 destroy the BM in glomerulus and lung.
44
What is osteoid, how is it made and degraded?
ECM in bone. It forms the organic, unmineralised portion of bone matrix It is made by osteoblasts and degraded by osteoclasts
45
what is the eCM in joints?
cartilage
46
what is the ECM in joints (cartilage) composed of ?
``` collagen type 2 aggrecan chondroitin sulphate Keratan sulpahte hyaluronic acid ```
47
give an example of a disease when elastin in ECM is damaged
Supravalvular Aortic Stenosis
48
give an example of a disease when Fibrillin-1 in ECM is damaged
Marfan Syndrome: skeletal, ocular & cardiovascular abnormalities
49
give an example of a disease when collagen in ECM is damaged
Ehlers-Danlos Syndrome: joint & skin abnormalities e.g. hypermobility, stretchy skin
50
give an example of a disease when keratin sulphate in ECM is damaged
Macular corneal dystrophy: corneal transparency
51
give an example of a disease when perlecan in ECM is damaged
Dyssegmental dysplasia Silverman-Handmaker : severe form of neonatal lethal dwarfism

Phase 1: weeks 1-5 (23 decks)

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