L23 Extracellular Matrix Flashcards

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1
Q

How much of our body is cells?

A

50%

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2
Q

Name some specialised ECM and their features

A
  1. Bone - Calcified
  2. Hair - Fibrous and loose
  3. Tendons - Tensile strength
  4. Skin - Elastic.
  5. Cornea - Transparent.
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3
Q

What are the features of ECM (Extracellular Matrix)?

A
  1. They have an extracellular meshwork of proteins and hydrated macromolecules.
  2. They regulate migration, tissue integrity, cell shape, proliferation and differentiation.
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4
Q

What are the types of ECM and give examples? (Check)

A
  1. Fibrous proteins - Collagen and elastin
  2. Adhesion proteins - Fibronectin and laminin
  3. Hydrated macromolecules - Glycosaminoglycan (Gags) and proteoglycans ( protein + Gags).
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5
Q

What is the main ECM component?

A

Collagen.

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6
Q

Describe the structure of Collagen

A
  1. They have 20 to 40 variants of collagen
  2. A triplet of Glycine-proline-hydroxyproline is repeated and form a long chain.
  3. They have a triple helix (3 polypeptide chains wind around each other)
  4. They have three alpha chains.
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7
Q

Where is collagen produced from in the body?

A

Fibroblasts and epithelial cells.

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8
Q

Give one collagen defect and what it causes

A
  1. Ehlers-danlos Syndrome (affects body’s connective tissues) - vascular form and arterial rupture.
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9
Q

Why does collagen needs to be synthesised? (Check)

A

Collagen undergoes hydroxylation to need to form a strong structure instead of it being comprimised.

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10
Q

What happens when elastin is stretched?

A

When elastin is stretched, it becomes deformed because its flexibility and recoil is essential for organs such as lungs and skin

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11
Q

How much percentage of elastin is in Aorta?

A

50% dry weight

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12
Q

What is elastin formed from? (Check)

A

Elastin is synthesised from Tropoelastin using Lysyl oxidase.

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13
Q

What is Fibrillin?

A

Fibrillin is a glycoprotein that plays a crucial role in the formation and structure of connective tissues throughout the body.

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14
Q

What does elastin laid down over microfibril scaffold of fibrillin cause? (Check)

A

Marfan syndrome which extends bones, eyes and causes aortic rupture.

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15
Q

What are GAG’s Glycosaminoglycans?

A
  1. They are Disaccharide Chains.
  2. 70-200 units long
  3. Highly Charged (polar)
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16
Q

Give some examples of GAG’s Glycosaminoglycans?

A
  1. Proteoglycan - 80 saccharides. 95% sugar.
  2. Glycoprotein - 15 Saccharides. 60% sugar.
17
Q

What are Hyaluronan complexes?

A

Hyaluronan complexes are mixtures of different molecular weight forms of hyaluronic acid. Hyaluronic acid (HA) is a naturally occurring substance found in the body, particularly in the skin, joints, and eyes.

Aggrecan

18
Q

What are the roles of Hyaluronan complexes?

A

It plays a crucial role in hydration, tissue repair, and lubrication

19
Q

Name a few adhesion glycoproteins

A

Laminin, Fibronectin

20
Q

What are integrin in Laminin?

A

Integrins is the part of the laminin which is responsible for adhesion.

21
Q

What are Fibronectin?

A

Fibronectin contains specific domains that bind to integrity receptors on cell surfaces. This interaction promotes cell adhesion and anchors cells to the ECM

22
Q

What are the features of integrin?

A
  1. Bind matrix through divalent cations (+2 charge)
  2. Removal of cations causes cells to detach.
  3. They from dimeric proteins.
  4. They have a transmembrane for signalling.
23
Q

What is the role of focal adhesions?

A
  1. Transmembrane receptors recieve signals.
  2. Connection of cytoskeleton.
  3. Signalling
24
Q

What are the knockouts of focal adhesion proteins?

A
  1. b1-integrin
  2. a5-integrin
  3. Fibronectin
  4. Talin
25
Q

What does focal adhesion knockouts cause?

A

Embryonic lethality.

26
Q

Name some integrin related defects (do more)

A
  1. αIIbβ3-integrin defect.
  2. β2-integrin
27
Q

What are the symptoms of αIIbβ3-integrin defect?

A
  1. Platelets bind fibrinogen to clot blood
  2. Glanzmann’s thrombasthenia
  3. Bleeding gums and nose bleeds
28
Q

What are the effects of b2 integrin?

A
  1. Leukocyte Adhesion Deficiency (LAD) syndrome
  2. Impaired expression
  3. Recurrent bacterial infections