Connective Tissue Flashcards

1
Q

3 cell types responsible for making connective tissue matrix

A

fibroblasts
chondrocytes
osteocytes

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2
Q

fibers (collagen, elastin, fibronectin, etc) in a hydrated gel (glycosaminoglycans + H2O) and growth factors / support proteins

A

matrix

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3
Q

How does ECM regulate cell behavior?

A

Cells adhere to and migrate differently on different ECM
ECM can bind and regulate growth factors
**ECM can itself act like growth factors. Cell have receptors for, and respond to ECM.

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4
Q

Densely packed collagen fibers, somewhat wavy but mostly parallel. Scanty fibroblasts wedged between fibers.
(tendons and ligaments)

A

Dense regular connective tissue

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5
Q

Dense collagen fibers, closely packed, but running in all directions
Few cells
(dermis)

A

Dense irregular connective tissue

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6
Q

Fewer and thinner collagen fivers, more space (watery gel) between
More cells
(hypodermis, lamina propria)

A

Loose (areolar) connective tissue

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7
Q

Special term for loose CT beneath a mucous (moist) epithelium - GI tract, respiratory, etc.

A

Lamina propria

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8
Q

Collage fibers are made of densely packed bundles of collagen ________ that cannot be resolved with a light microscope

A

Fibrils

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9
Q

Form 67nm banding pattern
About 50-150 nm in diameter
About 100 make bundles that are fibers

A

Collagen fibrils

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10
Q

In a collage triple helix, three collagen _______ chains are wrapped around each other, and support each other in a near maximally extended form.

A

alpha

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11
Q

Describe a collagen molecule

A

trimer
very long, thin
very limited stretch
rigidity of plexiglass

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12
Q

Characteristic signature of collagen chain

A

Every third aa is glycine
Gly needs to be in the center when three alpha chains come together
Closely packed center

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13
Q

Protein-protein bonds are _____________ and reversible (hydrophobic bonds)

A

non-covalent

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14
Q

The strength of the bond is roughly proportional to the ____________ of the contact interface.

A

surface area

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15
Q

Why is collagen made with a 67nm staggered overlap?

A

Increase surface area and strength

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16
Q

What is the hierarchy of collagen substructures from molecules to tendon?

A
Alpha chain 
Collagen molecule
Fibril (staggered overlap) 
Fiber
Tendon
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17
Q

How are fibrils and fibers stalinized once non-covalently self-assembled?

A

Intermolecular covalent crosslinks

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18
Q

How is triple helix assembly lined up and fibril formation prevented until properly distributed in ECM?

A

Propeptides

allow for assembly, but block fibril formation due to bulkiness

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19
Q

List the steps for collagen processing and assembly that occur in the RER and golgi before secretion.

A
  1. Synthesis and translocation of pro alpha chain into RER
  2. Hydroxylation of selected pro and lys
  3. Assembly of C-propeptides of three alpha chains **aligns the alpha chains
  4. Folding of the triple helix, from C to N term. (only begins are C-propeptide fold)
  5. Formation of N-term propeptide finishes the procollagen molecule
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20
Q

List the steps in fibril assembly following secretion.

A
  1. The N and C term pro peptides block assembly of the secreted procollagen molecules.
  2. Two enzymes, procollagen N and C-peptidase cleave the pro peptides.
  3. Once the propetides are released, the collagen molecules are active for assembly. They spontaneously self assemble into long, 50 nm diameter fibrils. (staggered)
  4. The fibrils aggregate side to side to form fibers.
  5. Covalent crosslinks are added to stabilize fibrils and fibers.
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21
Q

Why are blood vessels affected before tendons during vitamin c deficiency (scurvy)?

A

Tendons turn over more slowly

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22
Q

Align three alpha chains prior to folding triple helix
Block assembly of fibrils until molecules are properly deposited in ECM
In non-fibrillar collagens (Type IV), used for assembly

A

Pro-collagen peptides

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23
Q

Possible combinations for three alpha chains in molecule

A
three different (3 genes)
three identical (one gene) - Type 1 
two identical, one different (2 genes) - Type II, IV
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24
Q

Type of collagen found in skin, tendon, bond ligaments, cornea, internal organs (most of body’s collagen)

A

Type I

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25
Type of collagen found in cartilage, intervertebral disc, notochord, vitreous body of eye
Type II
26
Type of collagen found in basement membrane, and specialized basement membrane of kidney (glomerular BM)
Type IV
27
Found between epithelium and CT, surrounding muscle cells, and between two cell layers in the kidney (filtration barrier)
Basement membrane (basal lamina)
28
3 components of basement membranes
Collagen type IV Laminin Proteoglycans
29
Froms a 2-D net (not fibrils) A major structural component of basement membranes Cell adhesion **Binds integrin receptors
Collagen type IV
30
Second major structural component of basement membranes Also functions for cell adhesion of overlying epithelium **Binds integrin receptors
Laminin
31
Proteins with positively charged carbohydrate chains | Regulate filtration, passage of growth factors, and proteins in basement membrane
Proteoglycans
32
Functions: Attachment of epithelial cells (integrins, col IV) Barrier to cell migration (for metastasis cells must cross) Barrier to large molecules (filtration barrier in kidney) Cell signaling (define pathway for cell growth, migration - nerve regeneration) Cell signaling (induce endothelial cells to form capillaries, great epithelial cells to form secretory follicles)
Basement membranes (basal lamina)
33
Disorder with mutations in a type IV collagen with three minor alpha chains (found in kidney BM) Kidney degernation and hearing loss
Alport's syndrome
34
Complex molecule with multiple cell adhesion and interaction sites (globular domains) 3 chains makes triple coiled-coil helix Associates with itself and Col Type IV in BM
Laminin
35
Mediate cell-matrix adhesion TM cell surface receptors (bind ECM on extracellular side and cytoskeleton on the inside) Integrate matrix and cytoskeleton Alpha and beta subunits Different ones can bind fibronectin, laminin, or fibrinogen on platelets etc.
Integrins
36
Integrins connect platelets through fibrinogen (cross-linked) to form the ________, which is the primary factor for hemostasis
Platelet plug
37
Bleeding disorder due to lack of platelet aggregation and clotting Caused by genetic defects in an integrin
Glanzmann's thrombasthenia
38
2 protein components of elastic fibers and sheets
Elastin | Fibrillin
39
Found in blood vessels, lungs, dermis, GI tract (wherever stretching is needed) Can stretch 2x and contract fully
Elastic fibers and fenestrated sheets Fenestrated = holes to permit diffusion of nutrients
40
70kDa protein extensively cross linked to to make a fiber | cross-linked subunits can stretch
Elastin
41
350 kDa protein makes one microfibrils may define orientation of elastin deposition dominant (-) mutations cause Marfan's syndrome (aortic aneurism likely)
Fibrillin
42
OMIM
Online Mendelian Inheritance in Man
43
All large arteries have _____. The amount deposited in arterial walls is highly regulated.
elastin
44
Cells that make collagen and organize the assembly of collagens fibrils in grooves and tunnels on their surface Define directionality of fibers Maintain attachments to collagen through integrins - have contractile activity that can pull fibers together
Fibroblasts
45
Unilocular fat cells Large fat droplet surrounded by a thin halo of cytoplasm Most don't show a cytoplasm
Adipocytes
46
Many small fat droplets in each cell Many mitochondria Uncouples oxidative phosphorylation (UCP1) Heat producing (more efficient than shivering) Prominent in hibernating animals, rodents, human infants Adults - cold response (less is associated with obesity)
Brown adipose tissue
47
Reside in CT near blood vessels Release granules containing histamine, heparin, and cytokines including platelet activating factor, eosinophil chemotactic factor Clock face nucleus
Mast cells
48
Glycosaminoglycan that prevents clotting of leaked plasma | Binds and regulates many proteins
Heparin
49
Causes blood vessels to dilate, leak plasma | Edema produces symptoms of hay fever
Histamine
50
Activated B - secretes Ab Abundant RER Pale Golgi Clock-face nucleus
Plasma cell
51
Phagocytes that kill parasites Granulocyte Bi-lobed nucleus
Eosinophil
52
Two mechanisms for dominant negative mutations
Haplo-insufficiency | Assembly poison
53
defective protein is dead other gene produces half the normal amount of good protein half is not enough
Haplo-insufficiency
54
if half of the protein molecules are defective, they poison the rest (pro collagen suicide and murder, bent collagen poisons the fibrils)
Assembly poison
55
defective collage alpha chain starts to make triple helix, but folding is blocked unfolded protein is destroyed - two associated good chains are also destroyed
Pro-collagen suicide and double murder
56
collagen triple helix folds, but with a kink during fibril assembly, causes fibril to branch a few bad molecules can poison the good
Bent collagen
57
Collagen that keeps globular pro-peptides to aid in assembly End to end Doesn't make fribrils
Collagen Type IV
58
RBC x MCV | Percent of blood that is RBCs (45%)
Hematocrit (HCT)
59
Noncellular component of blood, plus fibrinogen
Plasma
60
Noncellular component of blood, sans fibrinogen (without)
Serum
61
Structural protein largely responsible for biconcave shape of erythrocytes
Spectrin
62
Blood groups are due to
Post translational modifications - addition of sugar groups | Genetic difference is whether you have the transferase enzymes
63
Hemoglobinopathy Alpha or beta mutations Like, Sickle cell, is protective against malaria
Thalessemia
64
Why does hemoglobin need to be packaged into RBCs?
Kidney filtration | Sequester iron
65
Causes hemoglobinuria, tubular necrosis, death | Requires dialysis treatment
Hemolysis
66
Organ where RBCs have open circulation
Spleen
67
Found on endothelial surface to initiate leukocyte rolling
selectins
68
Found on endothelial surface to trigger tight binding (arrest) of leukocytes
ICAM
69
Found on leukocytes, interact with ICAM for tight binding
Integrins (LFA-1)
70
Diapedesis in between two cell or uses pseudopod to poke through
Paracellular diapedesis
71
Diapedesis through the cell as a form of phagocytosis
Transcellular diapedesis
72
Adult hematopoiesis is made in the bone marrow, but fetal is made in:
Liver, spleen
73
What is yellow marrow?
Found more as you get older, more fatty
74
Platelet precursor
Megakaryocyte