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BIOCHEMISTRY > Chapter 50 Extracellular Matrix > Flashcards

Chapter 50 Extracellular Matrix Flashcards

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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
Distribution of the type 16 collagen
Many tissues
26
Distribution of type 17 collagen
Hemodesmosomes
27
Type 18 collagen distribution
Call Jen close to basement membrane close structural Homologue of 15
28
Distribution of type 19 collagen
Rhabdomyosarcoma cells which are rare
29
Distribution of your type 20 collagen
Corneal epithelium
30
Distribution of your type 21 collagen
Many tissues
31
Distribution of your type 22 collagen
Tissue junctions including your cartilage synovial fluid hair follicle dermis
32
Distribution of your type 23 collagen
Transmembrane and shed forms
33
Distribution of your type 24 collagen
Developing cornea and bone
34
Distribution of your type 25 collagen
Brain
35
Distribution of your type 26 collagen
Testis and ovary
36
Distribution of your type 27 cartilage
Embryonic cartilage and other developing tissues and cartilage in adults
37
Distribution of a collagen type 28
Basement membrane around Schwann cells
38
What is the repeating structure that is an absolute requirement for the formation of the triple helix
Gly- X-Y
39
The positions of X
PROLINE
40
THE POSITION OF Y
HYDROXYPROLINE
41
WHAT CONFERS RIGIDITY OF THE COLLAGEN MOLECULE
PROLINE AND HYDROXYPROLINE
42
HOW IS HYDROXYPROLINE FORMED?
By POSTTRANSLATIONAL HYDROXYLATION OF PEPTDE BOUND PROLINE RESIDUES CATALYZED BY ENZYME PROLYL HYDROXYLASE
43
What are the cofactors used in formation of hydroxyproline
Ascorbic acid and alpha ketoglutarate
44
Glycosylation side that is unique to collagen
O- glycosidic linkage
45
Assemble of triple helical units into lateral association
Fibrils
46
Structure of your fibrils
Quarter staggered alignment
47
Stabilization of your collagen fibers that happens within and between the triple helical helix
Covalent cross-link that are formed through the action of your lysyl oxidase
48
A copper independent enzyme that oxidative Lidia minutes the amino groups of certain lysine and hydroxylation residue
Lysyl oxidase
49
Products of the deamination of your lysyl oxidase
Aldehydes
50
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
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
What are your fibril forming collagen
I II III V XI XXIV XXVVII
52
What are your network like collagen
IV VIII X
53
What are your fibril associated collagens would interrupt the triple helices
ix Xii Xiv Xix Xx XXI XXII
54
What are your beaded filaments collagen
VI XXVI XXVIII
55
What is the only collagen that is anchoring fibrils
VII
56
Collagens that are transmembrane
XIII XVII XXII XXV
57
Multiplexins collagen
XV XVIII
58
Collagen are synthesized in the
Ribosomes
59
What is the precursor of the collagen
Preprocollagen
60
Preprocollagen is important because
It ccontains the signal sequence that directs the polypeptide chain into the lumen of endoplasmic reticlum
61
The precursor collagen enters the ER, what happens next
Hydroxylation of proline and lysine residues and glycosylation of hydroxylysines in the procollagen molecule
62
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
Extension peptides
63
What do extension peptides carry
Cysteine residues
64
What do amino terminal propeptide forms
Intrachain disulfide bonds
65
What do carboxyl terminal propepides form
Both intrachain and interchain disulfide bonds
66
What is cardinal principle in biosynthesis of collagen
Self assembly
67
After the secretion from the cell by the way of Golgi apparatus what happens next
Extracellular enzymes remove the extension peptides at the amino and carboxyl terminal ends
68
What are those extracellular enzymes that removes the extension peptides
Procollagen aminoproteinase Procollagen carboxyproteinase
69
After removing the extension peptides the procollagen is now called
Tropocollagen
70
Where do cleavage of propeptides occur
Crypts or folds in the cell membrane
71
What binds collagen fibers during aggregation and alters the kinetics of fiber formation in the pericellular matrix
Fibronectin
72
What type of interaction that serve to regulate the formation of collagen fibers and to determine their orientation in tissue s
The collagen specifically type IX is attached to glycoaminoglycan chain
73
Breakdown of collagen is increased when there is
Starvation and inflammatory states
74
Excessive production of collagen happens in
Hepatic cirrhosis
75
What is homotrimeric
Containing identical three pro-alpha chains such as in type 2 collagen
76
What is heterotrimeric
Containing three different pro-alpha chains such as in type 1
77
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.
Ehlers-Danlos syndrome
78
What are the types of collagen that has genetic defects in Ehlers-Danlos syndrome
Collagen 1, 3 and 5
79
The most common subtypes in Ehlers-Danlos Syndrome
Hypermobility Vascular Classical
80
What are the rare subtypes of Ehlers-Danlos syndrome
Kyphoscoliosis Arthrochalasis Dermatosparaxis
81
What is the most serious subtype because of the tendency for spontaneous rupture of arteries or the bowel reflecting abnormalities in type III collagen
Vascular subtypes
82
What do patients with kyphoscoliosiss exhibit
Progressive curvature of the spine and tendency to ocular rupture due to deficiency of lysy hydroxylase
83
What is lacking or defect in patients with abnormal thin, irregular collagen fibrils resulting in dermatosparaxus manifested by marked fragile and sagging skin
Deficiency of procollagen N-proteinase (ADAM metallopeptidase with thrombospondin type 1 motif
84
What is other term for heredidtary nephritis
Alport syndrome

BIOCHEMISTRY (3 decks)

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