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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
Q

Elastin fibers are found in:

A

the lungs (inhalation and exhalation)
tunica media of aorta

26
Q

Monomers of elastin

A

tropoelastin
13 different isomers

27
Q

Fibrillin Functions

A

formation of elastin fibers
sequesters TGFbeta
structural integrity of aortic wall and suspensory ligament of lens

28
Q

Type 1 Collagen
is
found
in:

A

Bone
Skin
Tendons
Dentin
Fascia
Cornea
LATE wound repair

Mnemonic: bONE

29
Q

Type II Collagen

A

Cartilage
Vitreous body
Nucleus pulposis

Mnemonic: carTWOlage

30
Q

Type III Collagen

A

Reticulin
skin
blood vessels
uterus
fetal tissue
EARLY wound repair

31
Q

Type IV Collagen

A

Basement Membrane
Lens

Mnemonic: Type IV, under the floor

32
Q

Elastin fiber components

A

2 alternating hydrophobic segments
Rich in glycine, proline, or other hydrophobic amino acid

33
Q

Cross-linking of Elastin

A

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
Q

Formation of elastin fibers from tropoelastin

A

tropoelastin deposited into fibrillin microfibrils –> fibulin crosslinks fibrillin and elastin subunits –> forms elastin fibers

35
Q

osteogenesis imperfecta causes

A

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
Q

OI Type I Cause

A

mildest form
Decreased COL1A1 (COL1A2) synthesis, but collagen molecules are NORMAL

normal genes, just not enough

37
Q

OI Symptoms

A

BITE
Bones: brittle, frequent fractures, spinal curvature
I (eyes): blue sclera
Teeth: lack of dentin
Ear: hearing loss (affects ossicles)
Tendons: loose joins

38
Q

Ddx for OI

A

child abuse

39
Q

OI Type II-IV cause

A

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
Q

Lethal type of OI

A

OI Type II
perinatally lethal

41
Q

Scurvy Cause

A

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
Q

scurvy population

A

young children, homeless, drug/alcohol addicts, >65
-reduced access to fresh fruit

43
Q

scurvy symptoms

A

ecchymosis (bruising/discoloration of skin)
bleeding gums
abnormal wound healing
weak joints

44
Q

Ehlers-Danlos Syndrome is a disorder of (fibrillar/network forming) collagen

A

fibrillar

45
Q

Classical EDS

A

Mutation in COL5A1 or COL5A2 gene

46
Q

Vascular EDS

A

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
Q

Symptoms of EDS

A

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
Q

Alport Syndrome type of collagen

A

Network forming collagen: Type IV
mutation in alpha3, alpha4, or alpha5 (x-linked) chains

49
Q

Alport Syndrome cause

A

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
Q

Alport syndrome symptoms

A

Progressive kidney disease
variable eye and ear defects

51
Q

Goodpasture Syndrome Collagen Affected

A

Type IV Collagen

52
Q

Goodpasture Syndrome pathophysiology

A

autoimmune disease

Goodpasture antibodies against the NC1 domain of collagen IV alpha3 of the glomerular and alveolar basement membranes

53
Q

Goodpasture Syndrome symptoms

A

Pulmonary: dyspnea, hemoptysis (coughing up blood)

Renal: hematuria, proteinuria, renal failure

54
Q

Marfan’s Syndrome collagen affected

A

Fibrillin-1 Mutation

55
Q

Marfan’s syndrome pathophysiology

A

reduced fibrillin-1 –> release of TGFbeta –> activates growth factor receptors –> growth and proliferation

56
Q

Alpha1-Antitrypsin Deficiency pathophysiology

A

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
Q

Alpha1-Antitrypsin deficiency symptoms

A

liver cirrhosis
early onset emphysema

Principles of Foundational Medicine (10 decks)

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