ecm Flashcards

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1
Q

what are the causes and symptoms of osteogenesis imperfecta?

A

defective collagen type 1 synthesis
point missense mutation
glycine –> bulkier amino acid
fragile bones, blue sclera, weak tendons, thin skin

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2
Q

what is the inheritance pattern of type 1 osteogenesis imperfecta?

A

autosomal dominant

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3
Q

what are the two types of osteogenesis imperfecta and how severe are they?

A

OI 1 - mildest and most common

OI 2 - very severe, de novo, lethal

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4
Q

what causes Marfan’s syndrome?

A

mutant, misfolded fibrillin which causes elastin to be too stretchy

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5
Q

what are the symptoms of Marfans?

A
tall
long arms and legs
loose joints
floppy cardiac valves
eye problems
aortic aneurysms
spider fingers
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6
Q

what is the inheritance pattern of Marfan’s?

A

autosomal dominant

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7
Q

explain the cell migration model

A

actin filaments polymerise to change the shape of the cel

adhesion proteins attach at the leading edge

detach from trailing edge

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8
Q

what happens in cell migration on a flat surface with no ECM?

A

cell detachment and apoptosis

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9
Q

how do cancer cells impact cell migration and the ECM?

A

cleave cell adhesion molecules
break down ECM
growth factors and cytokines facilitate migration and metastasise

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10
Q

what are the functions of the ecm?

A
  • Functions as adhesive substrate
  • Provides structure
  • Presents growth factors to their receptor
  • Stores growth factors
  • Senses and transduces mechanical signals
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11
Q

what is in ECM/

A

o Collagens
o Elastin
o Glycoproteins
o Proteoglycans

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12
Q

how are macromolecules secreted and how do they change outside of cells?

A

secreted as small precursors and they polymerise once outside cells

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13
Q

describe the structure of collage?

A

triple helix made up of glycine and either proline or hydroxyproline (every 3rd amino acid is glycine)

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14
Q

how much of the total body mass is collagen?

A

25%

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15
Q

how many types of collagen are there?

A

19

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16
Q

what are the 2 main families of collagen?

A

o Fibril forming – Type I, II and III

o Non-fibrillar – the rest

17
Q

where is type 1 collagen found?

A
skin
tendon
bone
most loose/dense CT
ligaments
18
Q

where is type 2 collagen found?

A

hyaline cartilage

19
Q

where is type 3 collagen found?

A

lymphoid organs

20
Q

where is type 4 collagen found?

A

basement membrane (along with type 6)

21
Q

what is osteogenesis imperfecta?

A

brittle bone disease

22
Q

what are the genes affected in type 1 osteogenesis imperfecta?

A

 Chromosome 17, COL1A1 gene

 Chromosome 7, COL1A2 gene

23
Q

what is the mutation which causes osteogenesis imperfecta type 2?

A

new mutation - de novo

24
Q

where is elastin found?

A

blood vessels, lungs, ligaments and dermis of skin

25
Q

what is elastin made of?

A

glycine
proline
more valine than collagen

26
Q

why do elastic fibres need fibrillin?

A

to stop them stretching too far
stability
helps anchor fibres to other ECM proteins

27
Q

what effect does damage to fibrillin have on TGF-B?

A

causes more TGF-B to be released into the ECM so fibroblasts make more ECM

28
Q

what are glycoproteins?

A

proteins with oligosaccharides attached

29
Q

what are the roles of glycoproteins?

A

o Receptors on cell surface - Bacteria, viruses, toxins, hormones, other cells
o Strength and support to the ECM
o Slime layer of bacteria and flagella

30
Q

what are the 2 main glycoproteins that help with cell adhesion?

A

fibronectin

laminin

31
Q

what happens to your preoteoglycans as you get older?

A

o Smaller protein cores and fewer side chains
o Less water held  drier  CT more brittle and more likely to be damaged  less shock absorbency
o Seen in IV disc

32
Q

what can cause odema?

A

increased proteoglycan formation

33
Q

what are the 5 major protein families that cell adhesion molecules belong to?

A

– Cadherins – interact with similar molecules on the other cell
– Ig Super family – interact with similar molecules on the other cell
– Selectins - selects and attaches a cell to the ECM
– Mucins – will attach to selectins
– Integrins – work with fibronectin and laminin

34
Q

describe the basic principles of cell migration on a 2D surface?

A
  • polymerisation of actin filaments at leading edge gives a protrusive force
  • senses environment as it proves forward
  • moves in direction of growth factors
  • new cell adhesion molecules at the front
  • adhesion molecules detach from the back
  • • Forces produced by contractile network combined with actin filament and disassembly help to retract the trailing cell edge.
35
Q

describe cell migration on a 3d fibrillary ecm?

A
  • Cell has to force its way through scaffold of collagen and elastin fibres – not unidirectional movement
  • If ECM is nicely lined up like in dense regular CT, then the cell can just push itself in one direction
  • If dense irregular or loose CT then its more likely to have an amoeboid movement.
36
Q

what are the steps of leukocyte extravasion?

A

“chemoattraction”, “rolling adhesion”, “tight adhesion” and “(endothelial) transmigration” (amoeboid movement through basement membrane to get to ECM).

37
Q

how do cells migrate in cancer?

A
  • Cancer cells produce proteins which break down the cell adhesion molecules.
  • Loosens cancer cells within the tumour cells, allowing them to break off.
  • Go through rolling movement and can break down ECM holding cells together.
  • Degradation of ECM allows invasive cells to migrate into surrounding tissue and vasculature
  • Activation of growth factors and cytokines increases cell migration and likelihood of metastasis