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BIOCHEMISTRY > Chapter 50 Extracellular Matrix > Flashcards

Chapter 50 Extracellular Matrix Flashcards

1
Q

Connective tissue is also known as

A

Extracellular matrix

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2
Q

What are the three major classes of biomolecules of extracellular matrix?

A
  1. Structural proteins
  2. Specialized proteins
  3. Proteoglycans
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3
Q

What are two specialized form of ECM

A

Bone and cartilage

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4
Q

What is the major component of most connective tissue

A

Collagen 25%

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5
Q

What do you call those who have collagen like domains in their structures but are not classified as collagen

A

Noncollagen collagens

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6
Q

The structure of all collagen types

A

Triple helical structure

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7
Q

The triple helical structure of collagen is made up of

A

Three polypeptide chains subunits

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8
Q

What position does glycine can be found

A

Every third position of the triple helical portion of the alpha chains

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9
Q

The reason as to why the glycine residues can be found in every third position of the triple helical portion

A

The only amino acid small enough to be accommodated in the limited space in the central coree of the triple helix

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10
Q

Distribution of Type 1 collagen

A

Non-cartilaginous connective tissue such as bone, skin and tendon

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11
Q

Distribution of type 2 collagen

A

Cartilage and vitreous humor

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12
Q

Distribution of type 3 collagen

A

Extensible connective tissue such as skin, lung and vascular system

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13
Q

Distribution of type 4 collagen

A

Basement membrane

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14
Q

Minor component of type 1 collagen

A

Type 5 collagen

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15
Q

Distribution of type 6 collagen

A

Muscles and most connective tissue

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16
Q

Distribution of type 7 collafgen

A

Dermal-epidermal junction

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17
Q

Distribution of type 8 collagen

A

Endothelium and other tissue

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18
Q

Distribution of type nine collagen

A

Tissue containing collagen 2

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19
Q

Distribution of type 10 collagen

A

Hypertrophic cartilage

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20
Q

Distribution of type 11 collagen

A

Tissues containing collagen two

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21
Q

Distribution of type 12 collagen

A

Tissues contain collagen type one

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22
Q

Distribution of collagen type 13

A

Neuromuscular junction

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23
Q

Distribution of type 14 collagen

A

Tissues contain collagen type one

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24
Q

Distribution of collagen type 15

A

Associated with collagen close the basement membrane including the eye muscle and micro vessels

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25
Q

Distribution of the type 16 collagen

A

Many tissues

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26
Q

Distribution of type 17 collagen

A

Hemodesmosomes

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27
Q

Type 18 collagen distribution

A

Call Jen close to basement membrane close structural Homologue of 15

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28
Q

Distribution of type 19 collagen

A

Rhabdomyosarcoma cells which are rare

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29
Q

Distribution of your type 20 collagen

A

Corneal epithelium

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30
Q

Distribution of your type 21 collagen

A

Many tissues

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31
Q

Distribution of your type 22 collagen

A

Tissue junctions including your cartilage synovial fluid hair follicle dermis

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32
Q

Distribution of your type 23 collagen

A

Transmembrane and shed forms

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33
Q

Distribution of your type 24 collagen

A

Developing cornea and bone

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34
Q

Distribution of your type 25 collagen

A

Brain

35
Q

Distribution of your type 26 collagen

A

Testis and ovary

36
Q

Distribution of your type 27 cartilage

A

Embryonic cartilage and other developing tissues and cartilage in adults

37
Q

Distribution of a collagen type 28

A

Basement membrane around Schwann cells

38
Q

What is the repeating structure that is an absolute requirement for the formation of the triple helix

A

Gly- X-Y

39
Q

The positions of X

A

PROLINE

40
Q

THE POSITION OF Y

A

HYDROXYPROLINE

41
Q

WHAT CONFERS RIGIDITY OF THE COLLAGEN MOLECULE

A

PROLINE AND HYDROXYPROLINE

42
Q

HOW IS HYDROXYPROLINE FORMED?

A

By POSTTRANSLATIONAL HYDROXYLATION OF PEPTDE BOUND PROLINE RESIDUES CATALYZED BY ENZYME PROLYL HYDROXYLASE

43
Q

What are the cofactors used in formation of hydroxyproline

A

Ascorbic acid and alpha ketoglutarate

44
Q

Glycosylation side that is unique to collagen

A

O- glycosidic linkage

45
Q

Assemble of triple helical units into lateral association

A

Fibrils

46
Q

Structure of your fibrils

A

Quarter staggered alignment

47
Q

Stabilization of your collagen fibers that happens within and between the triple helical helix

A

Covalent cross-link that are formed through the action of your lysyl oxidase

48
Q

A copper independent enzyme that oxidative Lidia minutes the amino groups of certain lysine and hydroxylation residue

A

Lysyl oxidase

49
Q

Products of the deamination of your lysyl oxidase

A

Aldehydes

50
Q

What are the six collagen types that do not form fibrils because there is interruptions of the triple helix with stretches of protein locking the repeat sequence

A
  1. Network like collagens
  2. Fibril associated collagens with interrupted triple helices (FACITS)
  3. Beaded filaments
  4. Anchoring fibrils
  5. Transmembrane collagens
    6.multiplexins
51
Q

What are your fibril forming collagen

A

I
II
III
V
XI
XXIV
XXVVII

52
Q

What are your network like collagen

A

IV
VIII
X

53
Q

What are your fibril associated collagens would interrupt the triple helices

A

ix
Xii
Xiv
Xix
Xx
XXI
XXII

54
Q

What are your beaded filaments collagen

A

VI
XXVI
XXVIII

55
Q

What is the only collagen that is anchoring fibrils

A

VII

56
Q

Collagens that are transmembrane

A

XIII
XVII
XXII
XXV

57
Q

Multiplexins collagen

A

XV
XVIII

58
Q

Collagen are synthesized in the

A

Ribosomes

59
Q

What is the precursor of the collagen

A

Preprocollagen

60
Q

Preprocollagen is important because

A

It ccontains the signal sequence that directs the polypeptide chain into the lumen of endoplasmic reticlum

61
Q

The precursor collagen enters the ER, what happens next

A

Hydroxylation of proline and lysine residues and glycosylation of hydroxylysines in the procollagen molecule

62
Q

Procollagen molecules contains. A polyppetide extensions of 20 to 35 kda at both amino and carboxyl terminal ends which are not present in mature collagen

A

Extension peptides

63
Q

What do extension peptides carry

A

Cysteine residues

64
Q

What do amino terminal propeptide forms

A

Intrachain disulfide bonds

65
Q

What do carboxyl terminal propepides form

A

Both intrachain and interchain disulfide bonds

66
Q

What is cardinal principle in biosynthesis of collagen

A

Self assembly

67
Q

After the secretion from the cell by the way of Golgi apparatus what happens next

A

Extracellular enzymes remove the extension peptides at the amino and carboxyl terminal ends

68
Q

What are those extracellular enzymes that removes the extension peptides

A

Procollagen aminoproteinase
Procollagen carboxyproteinase

69
Q

After removing the extension peptides the procollagen is now called

A

Tropocollagen

70
Q

Where do cleavage of propeptides occur

A

Crypts or folds in the cell membrane

71
Q

What binds collagen fibers during aggregation and alters the kinetics of fiber formation in the pericellular matrix

A

Fibronectin

72
Q

What type of interaction that serve to regulate the formation of collagen fibers and to determine their orientation in tissue s

A

The collagen specifically type IX is attached to glycoaminoglycan chain

73
Q

Breakdown of collagen is increased when there is

A

Starvation and inflammatory states

74
Q

Excessive production of collagen happens in

A

Hepatic cirrhosis

75
Q

What is homotrimeric

A

Containing identical three pro-alpha chains such as in type 2 collagen

76
Q

What is heterotrimeric

A

Containing three different pro-alpha chains such as in type 1

77
Q

Is is a disease formerly known as Cutis Hyperplasia whose principal clinical features are hyperextensibilty of the skin, abnormal tissue fragility and increased joint mobility.

A

Ehlers-Danlos syndrome

78
Q

What are the types of collagen that has genetic defects in Ehlers-Danlos syndrome

A

Collagen 1, 3 and 5

79
Q

The most common subtypes in Ehlers-Danlos Syndrome

A

Hypermobility
Vascular
Classical

80
Q

What are the rare subtypes of Ehlers-Danlos syndrome

A

Kyphoscoliosis
Arthrochalasis
Dermatosparaxis

81
Q

What is the most serious subtype because of the tendency for spontaneous rupture of arteries or the bowel reflecting abnormalities in type III collagen

A

Vascular subtypes

82
Q

What do patients with kyphoscoliosiss exhibit

A

Progressive curvature of the spine and tendency to ocular rupture due to deficiency of lysy hydroxylase

83
Q

What is lacking or defect in patients with abnormal thin, irregular collagen fibrils resulting in dermatosparaxus manifested by marked fragile and sagging skin

A

Deficiency of procollagen N-proteinase (ADAM metallopeptidase with thrombospondin type 1 motif

84
Q

What is other term for heredidtary nephritis

A

Alport syndrome

BIOCHEMISTRY (3 decks)

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