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Principles of Foundational Medicine > Collagen and Elastin (Sayner) > Flashcards

Collagen and Elastin (Sayner) Flashcards

1
Q

4 basic tissues of organs

A

Muscle
Connective
Epithelial
Nerve

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2
Q

Cells that secrete and organize the ECM

A

End in suffix “-blast”
i.e. fibroblasts, osteoblasts

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3
Q

Components of the extracellular matrix

A

Protein fibers (collagen, elastin)
Basement membrane
Adhesive proteins and large sugar molecules

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4
Q

Functions of connective tissue

A
  1. Mechanical support- resists tension and compression
  2. Organizes tissues
  3. Generates immune response
  4. Metabolic support- growth factors, hormones, high energy lipids
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5
Q

Fibrillar collagen types

A

Class: Fibrillar
Types: I, III, V

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6
Q

Network forming collagen

A

Class: basement membrane
Type: IV

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7
Q

Fibrillar collagen disorders

A

Osteogenesis Imperfecta
Scurvy
Ehler-Danlos

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8
Q

Network forming collagen disorders

A

Alport Syndrome
Goodpasture Syndrome

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9
Q

Disorders of elastin

A

Marfan’s Syndrome
Alpha1 Antitripsin Deficiency

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10
Q

heterotrimer

A

different types of alpha chains within collagen molecule
Ex: type 1 collagen: COL1A1 + COL1A2
-hundreds of repeats: very rigid

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11
Q

homotrimer

A

only one gene product makes up
Ex: Type III Collagen: COL3A1
amino acids interrupt the repeats– more flexible, forms networks

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12
Q

Structure of collagen alpha chain

A

Every third amino acid is Glycine
(Gly-X-Y)n

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13
Q

(Gly-X-Y)n

X and Y are frequently which amino acids?

A

Proline and Lysine

Mutations in Gly, Pro, and Lys can disrupt formation of collagen

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14
Q

Basement Membrane

A

secreted by endothelial and epithelial cells
separate the underlying tissue

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15
Q

When does a carcinoma become invasive?

A

If basement membrane is breached
Breached vessel = metastisis

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16
Q

Basement Membrane collagen type

A

Collagen IV molecules

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17
Q

Collagen IV molecules

A

6 different alpha chains forming unique expression patterns

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18
Q

Formation of basement membrane

A

protomers assemble into sheets –> alternating sheets of collagen IV and laminin interconnected by multi adhesive proteins –> junctions anchor cells into laminin and fibronectin within the basement membrane

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19
Q

Elastin fibers function

A

resilience and recoil after stretch

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20
Q

Elastin fibers structure

A

Interwoven with collagen to prevent overdistenstion

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21
Q

Elastin fibers are synthesized by:

A

Fibroblasts
Chondroblasts
Smooth muscle cells

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22
Q

Fibroblasts

A

skin, tendons, lungs

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23
Q

Chondroblasts

A

elastic cartilage (ear, nose, epiglottis)

24
Q

Smooth muscle cells

A

blood vessels, respiratory system, hollow organs

25
Elastin fibers are found in:
the lungs (inhalation and exhalation) tunica media of aorta
26
Monomers of elastin
tropoelastin 13 different isomers
27
Fibrillin Functions
formation of elastin fibers sequesters TGFbeta structural integrity of aortic wall and suspensory ligament of lens
28
Type 1 Collagen is found in:
Bone Skin Tendons Dentin Fascia Cornea LATE wound repair Mnemonic: bONE
29
Type II Collagen
Cartilage Vitreous body Nucleus pulposis Mnemonic: carTWOlage
30
Type III Collagen
Reticulin skin blood vessels uterus fetal tissue EARLY wound repair
31
Type IV Collagen
Basement Membrane Lens Mnemonic: Type IV, under the floor
32
Elastin fiber components
2 alternating hydrophobic segments Rich in glycine, proline, or other hydrophobic amino acid
33
Cross-linking of Elastin
LYSYL OXIDASE modifies lysine to desmosine and isodesmosine Lysine rich domains crosslink alpha helical segment through bi-, tri-, and tetrafunctional cross-links within mature elastin
34
Formation of elastin fibers from tropoelastin
tropoelastin deposited into fibrillin microfibrils --> fibulin crosslinks fibrillin and elastin subunits --> forms elastin fibers
35
osteogenesis imperfecta causes
Type I collagen deleterious mutation (90% of OI, types I-IV) Genes encoding cross-linking proteins and other accessory proteins required for fiber formation (10%)
36
OI Type I Cause
mildest form Decreased COL1A1 (COL1A2) synthesis, but collagen molecules are NORMAL normal genes, just not enough
37
OI Symptoms
BITE Bones: brittle, frequent fractures, spinal curvature I (eyes): blue sclera Teeth: lack of dentin Ear: hearing loss (affects ossicles) Tendons: loose joins
38
Ddx for OI
child abuse
39
OI Type II-IV cause
Deletions/mutations in the COL1A1/COL1A2 gene = ABNORMAL collagen molecules = more severe disease Shortened pro-alpha1 chain Replacement of glycine Altered C-propeptide (aggregation of C-terminus leading to folding of helix)
40
Lethal type of OI
OI Type II perinatally lethal
41
Scurvy Cause
vitamin c deficiency Proylyl- and lysyl-hydroxylase require vitamin C and iron to HYDROXYLATE ALPHA CHAIN --> without this, collagen fibers are weak due to lack of crosslinking
42
scurvy population
young children, homeless, drug/alcohol addicts, >65 -reduced access to fresh fruit
43
scurvy symptoms
ecchymosis (bruising/discoloration of skin) bleeding gums abnormal wound healing weak joints
44
Ehlers-Danlos Syndrome is a disorder of (fibrillar/network forming) collagen
fibrillar
45
Classical EDS
Mutation in COL5A1 or COL5A2 gene
46
Vascular EDS
Mutation in COL3A1 gene Collagen III has a core of type V, and type I helps cross link --> forms delicate network of reticular fibers reticular fibers surround: skin, liver, smooth muscles of arteries, GI tract, lymph nodes
47
Symptoms of EDS
joint hypermobility +/- dislocation late walking skin: hyperextensible, bruises easily tissues: abnormal bruising/bleeding unexplained blood vessel rupture, gastrointestinal perforation, organ rupture (vascular type) Abnormal scarring, splitting wounds
48
Alport Syndrome type of collagen
Network forming collagen: Type IV mutation in alpha3, alpha4, or alpha5 (x-linked) chains
49
Alport Syndrome cause
embryonic collagen form persists in adult -not as cross-linked, resistant to proteases LUNGS not affected: can still be fully functional with immature form of collagen
50
Alport syndrome symptoms
Progressive kidney disease variable eye and ear defects
51
Goodpasture Syndrome Collagen Affected
Type IV Collagen
52
Goodpasture Syndrome pathophysiology
autoimmune disease Goodpasture antibodies against the NC1 domain of collagen IV alpha3 of the glomerular and alveolar basement membranes
53
Goodpasture Syndrome symptoms
Pulmonary: dyspnea, hemoptysis (coughing up blood) Renal: hematuria, proteinuria, renal failure
54
Marfan's Syndrome collagen affected
Fibrillin-1 Mutation
55
Marfan's syndrome pathophysiology
reduced fibrillin-1 --> release of TGFbeta --> activates growth factor receptors --> growth and proliferation
56
Alpha1-Antitrypsin Deficiency pathophysiology
GLU342LYS; mutation in SERPINA1 AAT is synthesized in the liver (encoded by SERPINA1) and sent to the lung, where it protects elastin fibers from degradation by elastase
57
Alpha1-Antitrypsin deficiency symptoms
liver cirrhosis early onset emphysema

Principles of Foundational Medicine (10 decks)

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