extracellular matrix Flashcards

extracellular matrix (ECM): recall the definition and function of, the ECM; and explain the relationship between the ECM and connective tissues, recall examples of human disorders resulting from ECM pathology

1
Q

define extracellular matrix

A

complex network of carbs and proteins (fibrillar and non-fibrillar) surrounding cells in tissues which can interact with cell receptors

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2
Q

functions of extracellular matrix

A

mechanical support; influence growth, adhesion, differentiation

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3
Q

composition of connective tissue

A

epithelium → fibroblast → smooth muscle → fibroblast → epithelium

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4
Q

function of fibroblast

A

make extracellular matrix components; main support cell of connective tissue

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5
Q

basal lamina function

A

separates underlying connective tissue from epithelial cells

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6
Q

extracellular matrix components within connective tissue

A

collagens, multi-adhesive glycoproteins, proteoglycans

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7
Q

what type of collagen are located in the basement membrane

A

type IV

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8
Q

what type of collagen are located in the extracellular matrix

A

fibrillar collagen I-III

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9
Q

what glycoprotein is located in the basement membrane

A

laminin

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10
Q

what glycoprotein is located in the extracellular matrix

A

fibronectin

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11
Q

what proteoglycan is located in the basement membrane

A

perlecan

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12
Q

what proteoglycans are located in the extracellular matrix

A

dectin and versican

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13
Q

what affects the function of connective tissue regarding the extracellular matrix

A

composition and arrangement of extracellular matrix e.g. tendon and skin; bone; cartilage; vitreous humour (eye)

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14
Q

examples of gene mutations on extracellular matrix proteins

A

Marfan’s syndrome, osteogenesis imperfecta, Alport’s syndrome, epidermolysis bullosa, congenital muscular dystrophy

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15
Q

mutated protein in Marfan’s syndrome

A

fibrillin-1

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16
Q

mutated protein in osteogenesis imperfecta

A

type I collagen

17
Q

mutated protein in Alport’s syndrome

A

type IV collagen

18
Q

mutated protein in epidermolysis bullosa and congenital muscular dystrophy

A

laminin

19
Q

example of gene mutation on extracellular matrix catabolism

A

Hurler’s syndrome

20
Q

examples of fibrotic disorders due to excessive extracellular matrix deposition leading to replacement with collagen

A

cirrhosis, kidney fibrosis, lung fibrosis

21
Q

what causes kidney fibrosis

A

diabetic nephropathy

22
Q

what causes lung fibrosis

A

silicosis

23
Q

example of fibrotic disorders due to excessive loss of extracellular matrix

A

osteoarthritis

24
Q

osteoarthritis

A

loss of extracellular matrix (cartilage) in joints as aggrecan degrades and is lost to synovial fluid