Extracellular Matrix Flashcards

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1
Q

What is the extracellular matrix and what is its purpose?

A

It is the acellular component of tissue, a complex network of proteins and polysaccharides secreted locally by cells and remaining closely associated with these cells.
It provides structural, adhesive and biochemical signalling support

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2
Q

What are the two main components of the extracellular matrix?

A

Fibres

Ground substance

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3
Q

What type of fibres are present in the extracellular matrix?

A

Collagen

Elastin

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4
Q

What kind of molecules are present in ground substance?

A

Proteoglycans
Glycosaminoglycans
Glycoproteins

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5
Q

What are the possible functions of the extracellular matrix?

A

Providing mechanical and structural support
Providing tensile strength
Anchoring cells through cell-ECM junctions
Strongly influencing embryonic development
Providing pathways for cellular migration
Sequestering growth factors
Providing a residence for roaming phagocytic cells
Establishing and maintaining stem cell niches

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6
Q

How many different collagen types occur in vertebrates?

A

At least 28

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7
Q

What is the structure of collagen and what structural unit allows it to form the shape it does?

A

Collagen is comprised of three collagen polypeptides wound together into a triple helix. The gly-XY motif in the polypeptide chains allow the formation of the triple helix

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8
Q

How would collagen types one and two be described?

A

Fibrillar

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9
Q

What type of collagen could be described as sheet/network forming?

A

Type four

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10
Q

Which structural glycoprotein is required for the assembly of elastin unto functional fibres?

A

Fibrillin

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11
Q

What is ground substance?

A

An amorphous, colourless, gelatinous material which fills the spaces between fibres and cells

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12
Q

What are glycosaminoglycans and what is their function?

A

Stiff, hydrophilic negative molecules consisting of repeating disaccharide units, which are very good at holding water without distorting. They are resistant to compressive forces and form the carbohydrate component of poteoglycans.

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13
Q

What is the alternative name for a glycosaminoglycan?

A

Mucopilysaccharide

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14
Q

What is the alternative name for mucoproteins?

A

Proteolgycans

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15
Q

What is the structure if a proteoglycan?

A

Proteoglycans have a bottle-brush structure and are composed almost entirely of carbohydrate

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16
Q

Give examples of important structural glycoproteins in the extracellular matrix.

A
Collagen
Fibrillin
Fibronectin
Laminin
Entactin
Tenascin
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17
Q

How is procollagen converted to collagen?

A

By post-translational modification of glycosylation and hydroxylation

18
Q

What is elastin first synthesised as that then undergoes hydroxylation to form functional elastin?

A

Tropoelastin

19
Q

What is the general pathway of the synthesis of proteoglycans for the extracellular matrix?

A

Core protein synthesised on the rough ER
Disaccharide repeats added at the Golgi to form polysaccharide
Proteoglycan delivered to the ECM by exocytosis and assembled alongside other ECM components

20
Q

What molecules Carry out degradation of the extracellular matrix?

A

Proteases
Matrix metalloproteinases
Elastase

21
Q

In what situations can ECM remodelling be useful?

A

Wound repair
Embryogenesis
Angiogenesis

22
Q

What can result from excess activation fo matrix metalloproteinases?

A

Tumour cell invasion

23
Q

What are the three layers of the basement membrane in order from most superficial to most deep?

A

Lamina lucida
Lamina densa
Lamina fibroreticularis

24
Q

What is the function if the basement membrane if the skin?

A
Support
Bind to underlying connective tissue
Mediate signals between cells and connective tissue 
Determine cell polarity
Permit the flow of nutrients
Create a path for cell migration
Be a barrier to downward growth
25
Q

A disorder in the attachment of the epidermis to the basement membrane will result in what condition?

A

Epidermolysis bullosa

26
Q

The extracellular matrix of bone is composed primarily of what type of collagen?

A

Type I

27
Q

Cartilage is a type of ECM and is comprised of mainly what type of collagen?

A

Type II

28
Q

Supravalvular aortic stenosis can result from defects in which ECM component?

A

Elastin

29
Q

Marfan syndrome can result from defects in which ECM component?

A

Fibrillin 1

30
Q

Ehlers danlos syndrome can result from defects in which ECM component?

A

Collagen

31
Q

Macular corneal dystrophy can result from defects in which ECM component?

A

Keratan sukphate

32
Q

Silverman handmaker type of dyssegmental dysplasia can result for a defect in which ECM component?

A

Perlecan

33
Q

Where is type V collagen found?

A

Cornea and as a linker in the basement membrane

34
Q

Where is type III collagen found?

A

Liver
Bone marrow
Lymphoid organs
Granulation tissue

35
Q

Which GAGs is perlecan composed of?

A

Heparan sulphate

36
Q

Which GAGs is aggrecan composed of?

A

Keratan sulphate

Chondroitin sulphate

37
Q

Which GAGs is syndecan composed of?

A

Chondroitin sulphate

Heparan sulphate

38
Q

Which GAGs is decorim composed of?

A

Chondroitin sulphate

Dermatan sulphate

39
Q

Where is perlecan found?

A

The basement membrane

40
Q

Which glycoprotein is the primary organiser of the basement membrane?

A

Laminin