Extracellular environment Flashcards

1
Q

Where is type I collagen found

A
  • bone
  • skin
  • dentin
  • scar tissue
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2
Q

Where is type II collagen found

A
  • Cartilage
  • Vitreous body
  • nucleus pulposus
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3
Q

where is type III collagen found

A
  • blood vessels
  • skin
  • uterus
  • embryonic/fetal tissue
  • granulation tissue
  • Reticular fibers
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4
Q

Where is type IV collagen found

A

Basement membrane

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5
Q

What are the learning tools for types of collagen

A

“SCAB”

  • S: skin
  • C: Cartilage
  • A: Arteries
  • B: basement membrane

bONE: type 1

“a STRONG, SLIPPERY, STRETCHY BM”

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6
Q

What are the 2 common structures in preprocollagen alpha chains

A
  • glycine-proline-X

- glycine-X-hydroxyproline

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7
Q

In the synthesis of collagen, the hydroxylation of lysine and proline requires what

A

vitamin C

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8
Q

In the synthesis of collagen, what need to happen to the hydroxylated lysine to make procollagen (triple helix of alpha chains)

A

glycosylation

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9
Q

What happens to procollagen?

A
  • exocytosis

- cleavage of terminal ends —> tropocollagen

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10
Q

what happens to tropocollagen

A

-covalent crosslink to make collagen fibrils

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11
Q

Osteogenesis imperfecta (OI) type I is deficiency of what

A

type I collagen

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12
Q

Clinical features of OI type I

A
  • Multiple fractures, limb deformities
  • Blue sclerae
  • hearing loss
  • Dental abnormalities
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13
Q

describe OI type II

A

Death in utero or in the neonatal period (“perinatal lethal OI”)

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14
Q

Describe Classical Ehlers-Danlos syndrome (EDS)

A
  • affects mainly TYPE 4 and type 1 collagen

- hyperextensible skin and joint hypermobility

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15
Q

Describe hypermobility EDS

A

joint hypermobility WITHOUT hyperextensible skin

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16
Q

Describe vascular EDS

A
  • Defect in synthesis of TYPE 3 collagen

- Arterial rupture, hemorrhages, easy bruising, intracranial aneurysms

17
Q

Alport syndrome is defect of what

A

type 4 collagen

18
Q

Clinical features of Alport

A

-nephritis and kidney failure
-Hearing loss
-eye problems (e.g. cataracts, lenticonus)
“Can’t see, can’t pee, can’t hear high C”

19
Q

Defect in fibrillin (a component of elastin)

A

Marfan syndrome

20
Q

What amino acids make up elastin

A

Proline and glycine like collagen but NOT hydroxylated

21
Q

clinical features of Marfan syndrome

A
  • hyperelastic joints
  • disorders of heart valves and aorta
  • Tall stature
  • Long arms and legs; long fingers (“arachnodactyly”)
  • Pectus carinatum (anterior protrusion of the sternum)