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Basic Biochemistry > 49. Fibrous proteins > Flashcards

49. Fibrous proteins Flashcards

1
Q

most abundant protein in the body

A

collagen

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2
Q

collagen is stabilized by

A

hydrogen bonds

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3
Q

long stiff extracellular structure in which 3 polypeptides (alpha chains) each 1000 AA in length are wound around each other in a triple helix

A

collagen

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4
Q

most common form of collagen

A

type 1 collagen

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5
Q

collagen is rich in these AA

A

glycine

proline

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6
Q

this amino acid facilitates kinking

A

proline

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7
Q

collagen is formed by these cells

A

fibroblasts
osteoblasts
chondroblasts

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8
Q

type of collagen

Bone, skin, tendon

A

type I

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9
Q

type of collagen

cartilage, vitreous body, nucleous pulposus

A

type II

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10
Q

type of collagen

reticulin (skin), blood vessels

A

type II

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11
Q

type of collagen

basement membrane or basal lamina

A

type IV

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12
Q

type of collagen

beneath stratified squamous epithelium

A

type VII

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13
Q

type of collagen

dentin, fascia, cornea, late wound repair

A

type I

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14
Q

type of collagen

uterus, fetal tissue, granulation tissue

A

III

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15
Q

group of inherited disorders characterized by :
hyperextensibility of the skin
abnormal tissue fragility
inc joint mobility

A

Ehlers- Danlos syndrome

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16
Q

Defect in types I and V collagen

A

classical Ehlers- Danlos syndrome

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17
Q

defect in type III collagen

A

Hypermobility Ehlers- Danlos syndrome

Vascular Ehlers- Danlos syndrome

18
Q
  • joint hypermobility (less severe joint changes )

* severe skin abnormalities

A

classical

19
Q
  • joint hypermobility
  • skin abnormalities
  • OA
  • severe pain
A

hypermobility EDS

20
Q

most common EDS

A

hypermobility

21
Q

most serious EDS

A

vascular EDS

22
Q
  • fragile blood vessels and organs
  • small stature
  • thin and translucent skin and easy bruising
  • ÎÎ risk of intracranial aneurysms
A

vascular EDS

23
Q

brittle bone syndrome

A

osteogenesis imperfecta

24
Q

mutation in collagen genes that result to bones that is easily bend and fracture

A

osteogenesis imperfecta

25
Q

osteogenesis imperfecta

most common form

A

autosomal dominant with abnormal collagen type I

26
Q
  • hematuria (main presenting sign)
  • ocular lesions
  • hearing loss
  • eventually ESRD
A

alport syndrome

27
Q

multiple fractures
blue sclerae
hearing loss
dental imperfections

A

osteogenesis imperfecta

28
Q

the skin breaks and blisters as a result of minor trauma
Dystrophic form is due to mutations affecting structure of type VII collagen - which ancors basal lamina and collagen fibrils in the dermis

A

epidermolysis bullosa

29
Q

hydroxylation of collagen is a post transltional modification requiring

A

ascorbic acid

30
Q

deficiency leads to dec cross-linking of collaen fibers

A

scurvy

31
Q

sore spongy gums
loose teeth
poor wound healing
petechiae on skin and mucous membranes

A

scurvy

32
Q

characterized by kinky hair and growth retardation

dietary deficiency of copper

A

Menkes disease

33
Q

Menkes disease is a dietary deficiency of copper required by

A

lysyl oxidase

34
Q

connective tissue protein with rubber like properties responsible for extensibility and elastic recoil in tissues

A

elastin

35
Q

protein rich in proline mlysine, little hydroxyprolien and no hydroxylysine

A

elasin

36
Q

deposited into an irregular fibrillin scaffold cross-linked by desmosine

A

elastin

37
Q

mutation in fibrillin gene

A

marfan syndrome

38
Q

inheritance of marfan syndrome

A

Autosomal dominant

39
Q
  • taller and thinner than family members
  • dolichostenomella
  • arachnodactyly
  • aortic dilation (70-80%) and dissection
  • upward dislocation of the lens (ectopia lentis)
A

marfan syndrome

40
Q

inhibits proteolytic enzymes from hydrolyzing and destroying protins

A

alpha-1 antitrypsin

41
Q

pathophysiology of alpha-1 antitrypsin deficiency

A

elastase destroys alveolar walls -> emphysema

Basic Biochemistry (50 decks)

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