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UofG Medicine Question Bank 2020 > Extracellular Matrix > Flashcards

Extracellular Matrix Flashcards

1
Q

What are the 5 types of molecules found within ECM?

A

Fibrins -Elastin, Collagen,

Ground Substances- Proteoglycans, Glycosaminoglycans (typically hyaluronan), Glycoproteins

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2
Q

How many types of collagen are there?

A

28

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3
Q

What are the structural components of collagen?

A

3 collagen polypeptides secreted into ECM form triple helix

Formed of triple amino acid repeat (Glycine -X -Y)

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4
Q

Where is collagen type 1 found?

A

Skin, in tendons, and ligaments, and bone, fibrocartilage

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5
Q

Where is collagen type 2 found?

A

Hyaline cartilage

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6
Q

Where is collagen type 3 found?

A

Found in tissue, liver, lymphoid organs, granulation tissue (i.e. reticulin)

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7
Q

Where is collagen type 4 found?

A

Basement membrane

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8
Q

Where is collagen type 5 found?

A

Linker to basement membrane, and associates with collagen 1 in cornea

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9
Q

What is the purpose of elastin?

A

Enables shape recovery following deformation,

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10
Q

What is another name for collagen 3?

A

Reticulin

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11
Q

How is elastin assembled

A

Fibrous, assembly relies on presence of glycoprotein fibrillin which gets incorporated into the elastin fibre.

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12
Q

Where is elastin found?

A

In tissue (lungs, bladder and blood vessels)

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13
Q

What staining would you use when looking for elastin or collagen?

A

Van Gieson Stain

It would stain dark pink/purple and help differentiate between smooth muscle and

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14
Q

What are proteoglycan functions?

A

Highly negatively charged, therefore attracts water.

This retention supplies resistance to forces and hydration

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15
Q

What are Glycosaminoglycans (GAGs)and what do they form?

A

Chains of repeating disaccharide units.

Form carbohydrate part of proteoglycans

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16
Q

What Glycosaminoglycans (GAGs) are used in forming cartilage?

A

Chondroitin Sulphate

Keratan Sulphate

17
Q

What glycosaminoglycans (GAGs) form synovial fluid?

A

Hyaluronan (hyaluronic acid)- also found in other body tissues
Can bind 10,000 times its own weight.

18
Q

What glycosaminoglycans (GAGs) form basement membrane?

A

Heparan Sulphate

19
Q

What are proteoglycans?

A

Assembly of GAGs along a protein core (95% carbohydrate)

20
Q

What is the location and the GAGs that form Aggrecan?

A

Cartilage
Keratan Sulphates
Chondroitin Sulphates

21
Q

What is the location and the GAGs that form Perlecan?

A

Basement Membrane

Heparan Sulphate

22
Q

What is the location and the GAGs that form Syndecan?

A

Cartilage
Chondroitin Sulphate
Heparan Sulphate

23
Q

What is the location and the GAGs that form Decorin?

A

Widespread in connective tissue
Chondroitin Sulphate
Dermatan Sulphate

24
Q

What are the components of Aggrecan and its primary role in the body?

A

Assemble along Hyaluronan Core to form large negatively charged aggregate
In cartilage and joints, it interacts strongly with Collagen T2, to help resist deformation

25
Q

What are different types of glycoproteins involved with the ECM?

A

Fibrillin
Fibronectin
Laminin

26
Q

What is the functions of Fibrillin?

A

Controls deposition and orientation of elastin

27
Q

What is the function of Fibronectin?

A

Link basement membrane
Cell attachment to basement membrane
Organize ECM

28
Q

What is the function of Laminin?

A

Organize basement membrane
Interacts with the integrins (alpha-4, beta-4) found in hemidesmosomes, helping to attach cells to the basement membranes (i.e. dermal-epidermal junction, hemidesmosomes bind to laminins)

29
Q

What is a glycoprotein composed of?

A

Protein and carbohydrate chains.

i.e. Oligosaccharide chain with amino acid side- chains covalently linked to it

30
Q

What cell is largely responsible for the production of ECM?

A

Fibroblasts
Synthesize & secreted by fibroblast as fibrous proteins which undergo post-translational modifications, and are then assembled

31
Q

What are the fibrous proteins, posttranslational modifications, and proteins assembly of collagen?

A

Procollagen -> glycosylation and hydroxylation -> triple helix

32
Q

What are the fibrous proteins, posttranslational modifications, and proteins involved in the formation of elastin?

A

Tropoelastin -> hydroxylation -> fibrillin scaffolding, cross-linked fibres

33
Q

How can the ECM be targetted by invading pathogens?

A

Clostridium species and bacillus cereus secrete collagenases that break down ECM and aid in their invasion

34
Q

What role does ECM play in relation to tumour cells?

A

Tumour cells can secrete and recruit other cells that secrete Matrix Metalloproteases that can break down the ECM, aiding in invasion of tumour cells

35
Q

Supravalvular- Aortic Stenosis is a condition relating to ECM. Explain its cause and impact.

A

Caused autosomal dominant inheritance resulting in mutation of elastin, causing aortic valve defects (aorta needs lots of elastin).
This causes restrictions in blood flow and can be fatal

36
Q

Marfan Syndrome is a condition of the ECM. Explain its cause and impact.

A

Mutations in fibrillin-1 gene (needed for organization of elastin fibre), so mutations will impact structures dependent on fibrillin, causing reduction in elastin.
Causes skeletal, ocular, & cardiovascular abnormalities

37
Q

Alport syndrome is a condition of the ECM. Explain its cause and impact.

A

Hereditary kidney disease
Mutations in collagen 4 gene causes defect in type 4 collagen.
Structural abnormalities of glomerular basement membrane (and in eye and ear).
Dysfunction leads to proteinuria and haematuria (protein and blood in urine)

38
Q

Ehlers-Danlos syndrome is a condition of the ECM. Explain its cause and impact.

A

Collagen 1, 3, 5 mutations

Impacts connective tissue causing skin hyperextensibility, joint hypermobility,

39
Q

What are the more common proteoglycans and where are they found?

A

Perlecan is found in the basement membrane.

Aggrecan and Syndecan are found in cartilage, while Decorin is present in connective tissues

UofG Medicine Question Bank 2020 (42 decks)

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