Review Power Point Flashcards

1
Q

What 3 things make up ground substance?

A

Glycoproteins
Glycoseaminoglycans
Proteoglycans

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2
Q

Ground substance participates in binding cells to fibers of what?

A

Fibers of connective tissues

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3
Q

What is a glycoprotein?

A

A protein with 1 or more attached sugars

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4
Q

GAGs are a family of what?

A

Heteropolysacharides

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5
Q

What are 2 important characteristics of GAGs?

A

Very hydrated

Very negative

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6
Q

What makes up proteoglycans?

A

A protein core with many GAG side chains

Bottle brush

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7
Q

What makes and secretes the large majority of extracellular matrix?

A

Neighboring cells

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8
Q

What is the most common cell of connective tissue?

A

Fibroblasts

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9
Q

What cells secrete dentin?

A

Odontoblasts

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10
Q

What cells secrete enamel?

A

Ameloblasts

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11
Q

What is the structure of tropocollagen?

A

3 helical strands

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12
Q

How do tropocollagen fibrils arrange themselves?

A

In a staggered array of parallel bundles

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13
Q

What causes striations in tropocollagen?

A

The alignment of every 4th molecule

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14
Q

Tropocollagen crosslinks via what?

A

Lysinonorleucine

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15
Q

Production of lysinornorleucine requires which enzyme?

A

Lysyl amino oxidase

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16
Q

What cofactor is needed for lysyl amino oxidase?

A

Vitamin B6 (pyridoxine)

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17
Q

What types of collagens are found in interstitial connective tissue?

A

Types I, II, III, V, XI

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18
Q

What is the most abundant collagen type

A

Type I

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19
Q

Most connective tissues have which collagen type?

A

Type I

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20
Q

What are some examples of tissues containing type I collagen?

A
Bone
Tendon
Skin
Dentin
Ligament
Fascia
Arteries
Uterus
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21
Q

Type II collagen is a component of what type of cartilage?

A

Hyaline cartilage

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22
Q

Where is the 1 optometry correlation of type II collagen found?

A

In vitreous humor

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23
Q

Reticular fibers are made up of which collagen type?

A

Type III

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24
Q

Where are 3 places that type III collagen is found?

A

Skin
Arteries
Uterus

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25
Q

What is the predominant collagen type in periodontal ligament?

A

Type III collagen

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26
Q

T/F Type III collagen is often found in bone.

A

False

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27
Q

Which collagen type is the most abundant structural component of basement membranes?

A

Type IV

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28
Q

The lens has which collagen type?

A

Type IV

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29
Q

What makes type VIII collagen?

A

Endothelial cells

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30
Q

What makes type X collagen? When does this occur?

A

Chondrocytes

During endochondral ossification

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31
Q

Which collagen types are multiplexins?

A

Types XV and XVIII

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32
Q

Where are multiplexins found?

A

In basement membranes

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33
Q

Multiplexins have multiple triple helix domains and ___.

A

Interruptions

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34
Q

What is the precursor to tropocollagen?

A

Procollagen

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35
Q

What are the 6 steps of converting preprocollagen to procollagen?

A
  1. Signal peptide cleavage
  2. Registration peptides
  3. Hydroxylate proline and lysine
  4. Attach sugars to hydroxyproline and hydroxylysine
  5. Forme triple helix
  6. Package into vesicles
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36
Q

What are the 3 steps to convert procollagen to tropocollagen?

A
  1. Registration peptides cleaved off; forms insoluble tropocollagen
  2. Aggregation of tropocollagen forms fibrils
  3. Fibrils cross link
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37
Q

Which amino acid is involved in making desmosine cross links of elastin>

A

Lysine

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38
Q

The fibronectin network is secreted as what?

A

A dimer of 2 large monomers

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39
Q

Where are the 2 large monomers of the fibronectin network connected near?

A

C-terminals by disulfide bonds

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40
Q

What stabilizes the fibronectin network?

A

Transglutamase cross-linking

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41
Q

Unlike collagen, the fibronectin network assembly requires ___.

A

Cells

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42
Q

What does the fibronectin network do?

A

Helps organize extracellular matrix and mediate cell adhesion

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43
Q

Elastic fibers consist of an ___ component comprised of elastin.

A

Amorphous

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44
Q

Where are elastic fibers found?

A

Smooth muscle
Arteries
Elastic connective tissue

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45
Q

What are the triple stranded helices of elastin called?

A

Tropoelastin

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46
Q

Elastin is rich in which amino acids?

A

Glycine
Alanine
Lysine

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47
Q

Elastin fibers crosslink via what?

A

Desmosines

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48
Q

What is strange about the elastin helix?

A

It is not a true alpha helix

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49
Q

What makes up elastic fibers?

A

Fibrillins and other small proteins

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50
Q

Fibrillins are a family of large what?

A

Large glycoproteins consisting of EGF-like motifs

51
Q

Where are fibrillins found?

A

Arterial linings

Heart valves

52
Q

What “coats” elastin fibers?

A

Fibrillins

53
Q

Elastin and fibrillins are ubiquitous where?

A

In connective tissues

54
Q

What fibers are made first in fibrillin developments?

A

Oxytalan fibers

55
Q

Why is elastin insoluble?

A

Extensive cross-linking

56
Q

Laminins will bind to which ECM components?

A

Heparin
Type IV Collagen
Nidogen/entactin

57
Q

Complexes of laminins and ___ provide structural organization to basement membranes?

A

Heparan sulphate, as well as “other molecules”

58
Q

What 2 functions do laminins carry out in basement membranes?

A

Structural organization

Binding site for cells

59
Q

What is the shape of nidogen/enactin?

A

Dumbell shaped

60
Q

What is found on the ends of nidogen/enactin?

A

A laminin/collagen IV building domain on one end, and a self aggregating domain on the other

61
Q

What separates the two ends of nidogen/enactin?

A

A rod-like connecting domain

62
Q

What does nidogen/enactin do?

A

Dimerizes, then cross links laminin and type IV collagen

63
Q

Nidogen/enactin is a 50 KD glycoprotein found where?

A

In all basement membranes

64
Q

Fibulins contain __ EGF-like repeats?

A

9

65
Q

What do fibulins bind to?

A
Laminins
Nidogen
Fibrinogen
Collagen IV
Other fibulins
66
Q

What do fibulins do?

A

Thought to link cells to basement membranes

67
Q

Fibulins are which type of protein?

A

Glycoprotein

68
Q

In basement membranes, what forms an extensive, partially cross-linked network?

A

Type IV collagen

69
Q

What do laminin 1 and nidogen do in the structure of basement membrane?

A

Form a second extensive network

70
Q

What links the 2 major networks of basement membrane?

A

Nidogen binding to collagen IV

71
Q

What do proteoglycans do in the basement membrane structure?

A

Intermix with the networks

72
Q

What do fibulins bind in the basement membrane?

A

Nidogen
Laminin 5
Fibronectin
Collagen IV

73
Q

In the structure of basement membrane, fibronectin binds to what?

A

All collagen

74
Q

There is a log of ___-___ in the basement membrane.

A

Cross-linking

75
Q

How many amino acids are in osteocalcin?

A

50

76
Q

3 of the amino acids in osteocalcin are what?

A

Gamma-carboxyglutamate (Gla)

77
Q

The 3 gamma-carboxyglutamates in osteocalcin are formed post-translationally in what type of reaction?

A

Vitamin K dependent reaction

78
Q

What does osteocalcin bind to?

A

Binds calcium with high affinity

Adheres to hydroxyapatite crystals

79
Q

Where is osteocalcin found?

A

Bone
Dentin
Cementum

80
Q

What other role might osteocalcin play?

A

Regulating crystal growth (negative regulator)

81
Q

What forms nanochains?

A

Amelogenin

82
Q

What type of protein is amelogenin (fibrous or globular)?

A

Globular

83
Q

Amelogenin self-assembles into what?

A

Nanospheres

84
Q

What is the driving force for formation of parallel bundles of apaptite during enamel formation?

A

The self-assembly of amelogenin into nanochains (regulated crystal growth)

85
Q

What is the hallmark cell of acute inflammation?

A

Neutrophil

86
Q

What degrades elastin?

A

Neutrophil elastase

87
Q

Elastase is which type of enzyme?

A

Serine protease

88
Q

What inactivates elastase?

A

Alpha-1 antitrypsin

89
Q

What does a deficiency in alpha-1 elastase result in?

A

Emphysema

90
Q

What are the common symptoms of the group of 10 diseases in Ehlers-Danlos syndrome?

A

Fragile, hyperextensible skin

Hypermobile joints

91
Q

Most forms of EDS result from defects in which types of collagen?

A

Types I, II, and III

92
Q

What is the most sever type of EDS?

A

Type IV

93
Q

What is the gene mutation associated with type IV EDS?

A

col3A1 gene

94
Q

How is EDS type IV passed genetically?

A

Autosomal dominant

95
Q

The overextensible tissues caused by EDS type IV can lead to what?

A

Arterial rupture (sudden death)
Intestinal perforation
Rupture of uterus
Easily bruised, translucent skin

96
Q

What does the mutation of collagen alpha 1 (III) gene that causes EDS IV actually cause to occur?

A

Slow synthesis
Defective secretion
Increased susceptibility to degredation

97
Q

Osteogenesis imperfecta is a group of how many diseases?

A

8

98
Q

What causes osteogenesis imperfecta?

A

Defective chains assembling to form defective helices, then fibrils and fibers

99
Q

What is the major symptom of EDS types VII A, B, C?

A

Dislocation of major joints

100
Q

EDS types IVV A and B result from what mutation?

A

Mutations of collagen alpha 1 (I) or alpha 2 (I) gene

101
Q

A mutation in the collagen alpha 1 (I) or alpha 2 (I) gene eliminates what?

A

The cleavage site for collagen N-protease

102
Q

A lack of the cleavage site for collagen N-protease will cause what?

A

Incompletely processed collagens to assemble and form defective fibrils

103
Q

How is EDS VII C passed genetically?

A

Autosomal recessive

104
Q

EDS VII C (dermatosparaxis) results from a deficiency in what?

A

Collagen N-protease

105
Q

Blue sclera results from ___ ___ caused by what?

A

Osteogenesis imperfecta

Defective type I collagen

106
Q

What is the most common type of osteogenesis imperfecta?

A

Type I

107
Q

What are some symptoms of type I osteogenesis imperfecta?

A

Easily fractured bones
Loose joints
Blue sclera

108
Q

Which subtype of type I osteogenesis imperfecta results in dentinogenesis imperfecta?

A

Type IB

109
Q

What is the problem in osteogenesis imperfecta type I?

A

Normal collagen, but not enough of it

110
Q

What type of metabolism does cardiac muscle undergo?

A

Totally areobic

111
Q

Cardiac muscle oxidizes what 2 things?

A

Glucose

Ketone bodies

112
Q

How does cardiac muscle store energy?

A

As phosphocreatine

113
Q

After a heart attack, which enzyme spikes the highest and fastest?

A

Troponin

114
Q

What are the 3 enzymes to look for after a heart attack?

A

Lactate dehydrogenase
Troponin
Creatine kinase

115
Q

After a heart attack, which enzyme takes the longest to spike?

A

Lactate dehydrogenase

116
Q

What forms the globular “heads” of myosin?

A

Amino terminus

117
Q

In myosin, 2 heavy chains coil from where?

A

Carboxy terminus

118
Q

In myosin, light chains are associated with which region?

A

Head region

119
Q

In myosin, cleavage with trypsin and papain result in what?

A

Heavy and light meromyosin

120
Q

Which is the important calcium sensing troponin?

A

C troponin

121
Q

What does I troponin do?

A

Inhibits

122
Q

When a sarcomere contracts, which band gets smaller? Which band stays stable?

A

I band shortens

A band doesn’t change

123
Q

What are the boundaries of a sarcomere?

A

Z disk to Z disk