Lecture 4: ECM Flashcards
Extracellular Matrix
Marfan Syndrome is a defect in what molecule?
Fibrillin I
Fibril-Forming is what class of collagen?
Distribution?
Type I
Most connective tissue
What are proteoglycans?
Repeating sugar chains with some protein mixed in
Where is laminin found?
Role?
Basal lamina
Several domains with roles, forms networks
Think: “Networks”
Junctional Epidermolysis Bullosa (JEB) contains a defect where?
Lamina V – Basement membrane
What are the three binding sites on fibronectin?
- Proteoglycan
- Integrin
- Collagen
What collagen is affected in Osteogenesis Imperfecta?
Symptoms?
Collagen I
Mr. Glass
What collagen is affected in Dystrophic Epidermolysis Bullosa (DEB)?
Symptoms?
Collagen VII
Skin can slough off, blisters
What are the three stages (with times) of wound repair?
- Inflammatory (hours)
- Proliferative (hours-days-weeks)
- Remodeling (weeks-months)
What connects the two “trains” of fibronectin?
Dual disulfide bonds
Which collagen has two end caps?
What are they?
Collagen IV
N-terminal tetramer
C-terminal dimer
Anchoring Fibrils are what class of collagen?
Distribution?
Type VII
Attachments of basal laminae to underlying connective tissue
What molecule gives elasticity to tissues?
What is an example of this?
Fibrillin
Aorta flexibility to accodate changing volume
Nervous Tissue?
Conductive tissue that distributes signals to control bodily functions
What are main STRUCTURAL classes in the ECM?
Collagen, Proteoglycans, Fibrillin
What is an important role of fibronectin?
Blood clotting
Hint: Think “adhesive” proteins
What is the role of fibroblasts
Secrete proteins