Extracellular Matrix Flashcards

1
Q

what is the ECM?

A

non-cellular component present within all tissues and organs

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2
Q

what are the 2 main types of ECM?

A

interstitial CT matrix - surrounds cells and provides structural scaffolding for tissues
basement membrane - separates epithelium from surrounding stroma

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3
Q

what is the composition and function of ECM?

A

complex network of proteins and polysaccharides
provices structure, adhesive and biochemical signalling support

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4
Q

where is ECM found?

A

bone, tendons, cartilage, blood vessel walls, vitreous body of the eye, cornea, dermal skin layer,

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5
Q

what are the 3 classifications of ECM function?

A

provides mechanical/structural support
tensile strength
determines cellular microenvironment

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6
Q

what is interstitial CT made of?

A

fibrous proteins (collagen/elastin)
ground substance (proteoglycans, glycosaminoglycans, glycoproteins)

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7
Q

describe the structure of collagen in ECM

A

insoluble fibrous protein, 3 collagen polypeptides form a triple helix, Gly-X-Y repeat modifications occur

fibrillar (type 1/2) in skin, tendon and bone provide strength
sheet/network forming (type 4) in BM provides support/filtering

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8
Q

explain the structure of elastin in ECM

A

fibrous structural protein
abundant in tissues requiring stretch and recoil properties
assemply into functional fibres requires presence of structural glycoprotein fibrillin

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9
Q

describe ECM arrangement in CT

A

loose irregular CT - lymphoid tissue
dense irregular CT - dermis
specialised - dense regular (tendons/ligaments), bone, cartilage

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10
Q

describe ground substsance

A

amorphous, gel-like, non-fibrous substance surrounding cells

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11
Q

describe glycosaminoglycan structure

A

chains of repeating disaccharide units, carb components of proteoglycans
attracts water giving it gel/cushioning and hydrating properties
very hydrophilic

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12
Q

describe proteoglycan structure

A

protein core + GAGs = ‘bottle-brush’ structure
90-95% carbs (GAG)

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13
Q

give 3 examples of glycoproteins in ECM and their function

A

fibrillin - controls deposition/orientation of elastin
fibronectin - linker role in BM; organises ECM and participates in BM cell attachment
laminin - primary organiser in BM layer

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14
Q

explain the synthesis, PTMs and protein assembly of ECM fibrous proteins

A

collagen - synthesised as procollagen, PTM either glycosylation or hydroxylation, protein assembled in triple helix

elastin - synthesised as tropoelastin, PTM hydroxylation, protein assembled in fibrillin scaffolded cross linked fibres

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15
Q

explain the synthesis of ECM proteoglycans

A

synthesised on rER, addition of polysaccharide as disaccharide repeats in golgi, delivered to extracellular compartment by exocytosis, assembly with other ECM components

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16
Q

describe ECM remodelling

A

deposition -> degredation -> modification
(reversible)

17
Q

give an example of deregulated ECM remodelling

A

excess activation of MMPs can cause a tumour cell invasion

18
Q

explain ECM structure in epithelial tissue

A

the BM
thin, tough, sheet of ECM with a mat-like arrangement

19
Q

describe the 3 ways BM can act on cells

A

lies underneath cells (epithelial/endothelial cells)
surrounds cells (muscle fibres)
separate 2 sheaths of cells (kidney and glomerulus)

20
Q

what are the functions of BM?

A

support, binds to underlying CT, mediates signals between cells and CT, determines cell polarity, permits nutrient flow, cell migration path, downward growth barrier

21
Q

give an example of a BM disorder

A

cancer - once BM is breached, epithelial tumours regarded as malignant

22
Q

describe the ECM in bone

A

organic (30%) -> type 1 collagen (osteocalcin)
inorganic (70%) -> hydroxyapatite
ECM in bone - osteoid

23
Q

name the 3 functional cell types in bone

A

osteoblast - matrix production
osteoclast - matrix resorption
osteocyte - maintenance

24
Q

what does cartilage contain to become a proteoglycan?

A

aggrecan + hyalraunic acid

25
name 3 types of cartilage
hyaline cartilage, elastic cartilage, fibrocartilage
26
describe the structure of cartilage
synthesised by chondrocytes, contains: type 2 collagen chondroitin suplhate keratan sulphate hyalraunic acid
27
is cartilage vascular or avascular?
avascular
28
which type of cartilages contain collagen fibres
fibrocartilage (abundant) and hyaline (few)
29
what can over/under production of ECM cause?
over-production = fibrosis over-degredation = osteoarthritis
30
how is marfans disease caused by an ECM abnormality?
a fibrillin-1 gene mutation affects skin CT, bone, and vessels results in vision problems, heart defects, long/slender limbs, fingers and toes
31
what is alport syndrome and what is it caused by?
mutation in collagen 4 genes, BM in glomerulus becomes defective and causes blood/protein in urine
32
what is ehlers-danlos syndrome and what causes it?
due to collagen 1/3/5 mutations, results in abnormal collagen production resulting in hypermobility and stretchy, fragile skin