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DIT > 1H Foundations 8 - Extracellular environmant > Flashcards

1H Foundations 8 - Extracellular environmant Flashcards

1
Q

What protein is involved in transporting an endocytosed vesicle from the plasma membrane to the endosome?

A

Clathrin

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2
Q

Which metals are known to facilitate the generation of oxygen free radicals?

A

Iron and copper

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3
Q

Which tumor suppressor proteins prevent the progression of the cell into S-phase?

A

Rb and p53

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4
Q
  • Most abundant protein in the body
  • Extensively modified by posttranslational modification
  • Organises and strengthens extracellular matrix
A

Collagen

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5
Q

Most common (90%) type of collagen

A

Type 1

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6
Q

Where can you find type 1 collagen?

A

In hard things (bONE)

  • Bone
  • Skin
  • Tendon
  • Dentin
  • Fascia
  • Cornea
  • Late wound repair
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7
Q

Which type of collagen is defective in Osteogenesis imperfecta?

A

Type 1

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8
Q

Where can you type 2 collagen?

A

In slippery things (carTWOlage)

  • Cartilage (including hyaline)
  • Vitreous body
  • Nucleus pulposus
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9
Q

Where can you find type 3 collagen?

A

In bloody things

  • Blood vessels
  • Skin
  • Uterus
  • Fetal tissue
  • Granulation tissue
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10
Q

Which type of collagen is defective in Ehlers-Danlos?

A

Type 3 (threE D)

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11
Q

Where can you find type 4 collagen?

A

Basement membrane or basal lamina (under the floor)

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12
Q

In which disease is type 4 collagen defective?

A

Alport syndrome

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13
Q

Mnemonic for Collagen?

A
  1. Strong
  2. Slippery
  3. Bloody
  4. BM (bowl movement)
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14
Q

Where is the basic peptide of collagen synthesised?

A

Rough ER of fibroblasts

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15
Q

What is the name of the primary protein produced in the production of collagen?

A

Preprocollagen

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16
Q

1st step in synthesis of collagen?

A

Synthesis of preprocollagen alpha chains. Usually Gly-X-Y

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17
Q

2nd step in synthesis of collagen and what does it require?

A

Hydroxylation of lysine and proline. Vitamin C

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18
Q

No vitamin c leads to?

A

Scurvy

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19
Q

3rd step in synthesis of collagen?

A

Glycosylation of hydroxylated lysine. Procollagen is the result. It is a triple helix of 3 collagen alpha chains. Hydrogen and disulfide bonds are used.

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20
Q

4th step in synthesis of collagen?

A

Exocytosis of procollagen into extracellular space.

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21
Q

Problems forming triple helix in collagen synthesis leads to?

A

Osteogenesis imperfecta

22
Q

5th step in synthesis of collagen?

A

Proteolytic cleavage of disulfide-rich terminal regions of procollagen, transfrorming it into insoluble tropocollagen

23
Q

6th step in synthesis of collagen?

A

Crosslink tropocollagen molecules to make collagen fibrils (by Cu2+ containing lysys oxidase). Covalent bonds between lysine and hydroxylysine

24
Q

Problems in cross linking tropocollagen into collagen causes?

A

Ehlers-Danlos

25
Q

A scar with too much collagen deposition?

A

Keloid

26
Q

Treatment of Keloids?

A

Injection of glucocorticoids

27
Q

OI type 1

A
  • Autosomal dominant
  • Normal type 1 collagen but not enough
  • Multiple fractures
  • Blue sclerae
  • Hearing loss
  • Dental abnormalities
28
Q

OI type 2

A
  • Autosomal recessive

- Perinatal lethal OI

29
Q

Classical Ehlers-Danlos syndrome

A
  • Affects type 5 collagen (mainly)
  • Affects type 1 collagen
  • Hyperextensible skin
  • Joint hypermobility
30
Q

Vascular Ehlers-Danlos

A
  • Affects type 3 collagen
  • Arterial rupture
  • Hemorrhages
  • Easy bruising
  • Berry aneurysms
31
Q

Alport syndrome

A
  • Defect of type 4 collagen
  • Kidney disease
  • Hearing loss
  • Eye problems (Cataracts, Lenticonus)

Can’t see
Can’t pee
Can’t hear high C

32
Q

Where do you find Elastin?

A
  • Blood vessels
  • Alveoli
  • Larynx
  • Ligamentum flavum
33
Q

What is elastin made of?

A
  • Rich in Proline and glycine

- Non-hydroxylated

34
Q

How is elastin structured?

A

As tropoelastin. Held together by a matrix of fibrillin

35
Q

Where is fibrillin produced?

A

Fibroblast

36
Q

What’s the defect in Marfan syndrome?

A

It’s a defect in fibrillin

37
Q

Symptoms of Marfan

A
  • Hyperelastic joints
  • Problems with heart valves, aorta
  • Very tall
  • Long arms and legs
  • Arachnodactyly
  • Steinberg sign
  • Pectus carinatum (pigeon chest)
38
Q

What breaks down elastin?

A

Elastase

39
Q

What inhibits elastase?

A

alpha-1-antitrypsin

40
Q

Alpha-1-antitrypsin deficiency characteristics

A
  • Destruction of elastin in the alveoli
  • Emphysema
  • Liver problems (due to deficient a-1-a accumulating in liver)
41
Q

How are metalloproteases involved in angiogenesis?

A

They degrade the wall of existing vessels in order to facilitate branching

42
Q

What does VEGF do in existing vessels?

A

Signals to vascular endothelium to migrate towards place of need

43
Q

How is VEGF involved in angiogenesis when not branching from an existing vessel?

A

It can recruit from the bone marrow endothelial progenitor cells to the place og need and make new vessels that connect to the old vascular bed

44
Q

Cutaneous wound healing. 0-3 hours?

A

Hemorrhage and clotting

45
Q

Cutaneous wound healing. 12-24 hours?

A

Acute inflammation (PMNs) (Neutrophils)

46
Q

Cutaneous wound healing. 1-3 days?

A
  • Macrophage infiltration
  • Granulation tissue (fibroblasts and vascular endothelial cells)
  • Epithelialization
47
Q

Cutaneous wound healing. Weeks-months?

A

Collagen production (type 3 then type 1)

48
Q

What aa’s do you find a lot of in collagen?

A

Glycine, Proline, Hydroxyproline

49
Q

What aa’s do you find a lot of in Elastin?

A

Glycine and Proline

50
Q

What’s the role of vitamin C in collagen production?

A

Hydroxylation of lysine and proline

DIT (11 decks)

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