Extracellular Matrix Flashcards

1
Q

3 functions of ECM?

A

physical support
determine tissue properties (mechanical and physiochemical)
Growth, adhesion and differentiation of its tissues

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2
Q

3 process ECM is needed for?

A

.

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3
Q

Components of ECM (2)

A

.

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4
Q

4 components of connective tissue?

A

.

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5
Q

glycoprotein vs proteoglycan?

A

(see pic)

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6
Q

composition of a general collagen molecule?

A

3 alpha chain (homo/heterotrimer)
each chain ~1000 AAs long
left handed helix
(every 3rd AA=glycine)

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7
Q

how are covalent crosslinks formed intra/intermolecularly

A

.

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8
Q

Why dies scurvy lead to tissue instability (refer to collagen and vit c)

A

.

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9
Q

what are collagen alpha chain precursors called?

A

.

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10
Q

what is tropocollagen?

A

a single unit of collagen, used to form fibrils

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11
Q

What provides the tensile strength of collagen fibres?

A

The parallel bundle arrangement so tensile forces are resisted in one direction

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12
Q

What is EDS (Ehlers-Danlos syndromes)

A

collagen mutations lead to stretchy skin and loose joints

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13
Q

Do all collagens form fibrils? Give an example for your answer

A

Collagen type IV does not form fibrils : it is present in all basement membranes
but it can form dimers, tetramers and higher order complexes

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14
Q

Whats a basal lamina

A

basement membrane (pl. basal laminae)

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15
Q

Where are basement membranes found?

A

surround muscle, peripheral nerve and fat cells and underlie most epithelia (inc. kidney)

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16
Q

What is Alport syndrome?

A

collagen IV mutation = GBM abnormally split + laminated –> progressive loss of kidney function (and also hearing)

17
Q

Elastic fibres : role and components?

A

provide elasticity
collagen and elastin fibres interwoven
made of elastin core and microfibrils (made of fibrillin)

18
Q

Whats Marfan’s syndrome?

A

Fibrillin-1 mutation

skeletal, ocular and cardiovascular manifestations (aortic ruptures, arachnodactyly)

19
Q

Elastin components (2) and function

A

2 types of segments: hydrophobic + alpha helices (alanine and lysine rich –> good for cross linking)
elastin is a protein making elastic fibres

20
Q

what does ‘modular architecture’ mean?

A

.

21
Q

meaning of ‘multi-adhesive’ glycoproteins

A

binding various matrix components and cell-surface receptors.

22
Q

Laminin structure

A

heterortrimeric proteins made
of: α ,β and γ chain
very large proteins with each chain having a molecular weight of between 160 and 400 kDa

23
Q

Laminin function

A

multi-adhesive proteins

interact with a variety of cell surface receptors including integrins and dystroglycan

24
Q

Causes/symptoms of congenietal muscular dystrophy

A

absence of the α2 chain in laminin 2.

hypotonia (abnormally decreased muscle tension), a generalised weakness and deformities of the joints

25
Q

Fibronectins (function, components + what do they exist as)

A

.

26
Q

Roles of fibronectins (4)

A

regulating cell adhesion, cell migration (embryogenesis + tissue repair), wound healing, mechanical continuum of cytoskeleton

27
Q

What do integrin receptors do?

A

.

28
Q

What are the 4 families of proteoglycans?

A

BALerS

29
Q

What is a GAG chain?

A

.

30
Q

How does cartillage create a huge tensile strength

A

.

31
Q

What are the 4 main groups of GAG proteins?

A
  • Hyaluronan
    • Chondroitin sulfate and Dermatan sulfate
    • Heparan sulfate
  • Keratan sulfate
32
Q

Properties of hyaluroanan (5)

A
  • soft connective tissues
  • simple carb chain w no core protein
  • unsulfated
  • spun out directly from enzymes in plasma membrane (not golgi or RER)
  • can undergo high degree of polymerisation (occupy large vol)
33
Q

Aggrecan properties (5)

A
  • major part of cartilage ecm
  • highly sulfated (-ve charge)
  • lots of carboxyl groups (-ve charge)
  • retain large quatities of water (due to -ve charge)
  • give up water in compressive load but regain after load reduced (= resists compressive forces perfectly)
34
Q

What happens to ecm in osteoarthritis?

A

.

35
Q

How do fibrotic diseases arise?

A

an excessive production of fibrous connective tissue