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Dermatology
Biology of the Extracellular Matrix
Built from Dermatology, 5th Edition
What’s inside
10 sections · 89 slides
What the Extracellular Matrix Is
A structural scaffold that also talks to cells
- The extracellular matrix in the skin
- The ECM is more than scaffolding
- From molecule to tissue architecture
- Dermal extracellular matrix networks
- Components of the extracellular matrix
- Components of the extracellular matrix (ECM)
- Two families of ECM molecules
Collagens
28 proteins built on one triple-helix design
- The collagen family
- The collagen triple helix
- The collagen family of proteins
- The collagen family of proteins
- The collagen family of proteins (continued)
- The collagen family of proteins (continued)
- The collagen family of proteins (continued)
- Collagen biosynthesis: inside the cell
- Collagen biosynthesis pipeline
- Enzymes that build collagen
- Supramolecular assemblies of collagens
- Supramolecular assemblies of collagens
- Collagens of the dermis
- Fibrillar networks in human skin
- Collagen VI and collagen IV
- Collagens that hold skin layers together
- Collagens of the vascular basement membrane
Elastic Fibers
Elastin and the microfibril scaffold that shapes it
- What makes skin stretch and spring back
- Elastin
- Microfibrils
- Fibrillins anchor the microfibril network
- Microfibrils store growth factors
- Microfibril and elastic fiber assembly
- Regulation of transforming growth factor-beta activity
The Extrafibrillar Matrix
The water-holding gel between the fibril networks
- Beyond "ground substance"
- Glycosaminoglycans (GAGs)
- What a proteoglycan is
- Proteoglycans of the skin
- Versican: the dermis's water magnet
- Versican structure and aggregates
- Heparan sulfate proteoglycans
- Small leucine-rich proteoglycans (SLRPs)
- Laminins
- Other glycoproteins of the skin
- Glycoproteins of the skin
Functions of the Extracellular Matrix
Structural scaffold and cell-behaviour regulator
- Structural roles of the ECM
- The ECM regulates cell behaviour
- Integrins: the cell's ECM receptors
- Integrin signalling and mechanosensing
- TGF-beta: an ECM-stored growth factor
Ehlers-Danlos Syndrome
A family of connective-tissue diseases from ECM gene defects
- How ECM gene defects cause disease
- Ehlers-Danlos syndrome: collagen defects
- Classic EDS and minor collagens
- EDS from faulty collagen processing
- Dermatosparaxis
- EDS from faulty proteoglycan linking
- EDS grouped by molecular defect
- Selected genetic ECM diseases of the skin
- Selected genetic ECM diseases of the skin
- Phenotypes of genetic ECM defects
Other Heritable ECM Disorders
Cutis laxa, Marfan syndrome, PXE and epidermolysis bullosa
- Cutis laxa
- Genes behind cutis laxa
- Marfan syndrome
- Loeys-Dietz syndrome
- Other fibrillin-linked syndromes
- Pseudoxanthoma elasticum (PXE)
- Epidermolysis bullosa: dystrophic type
- Epidermolysis bullosa: junctional type
- Other hereditary matrix diseases
ECM in Autoimmune Disease and Aging
When the immune system, or time, attacks the matrix
- ECM components as autoantigens
- ECM components as targets in autoimmune diseases
- Autoantibody targets in practice
- Skin aging and the ECM
- Scurvy and fibrotic disorders
Animal Models
Mice, rats, dogs and sheep that recreate human ECM disease
- Why animal models matter here
- What animal models have taught us
Molecular Therapies
Turning ECM biology into treatment for skin fragility
- Cytoskeletal organisation in a dermal fibroblast
- Microfibrils in the papillary dermis
- A landmark gene-therapy success
- Topical gene therapy for dystrophic EB
- Cell and protein-based therapies
- Repurposed drugs and future directions
- Key takeaways
- References
- References (continued)
- References (continued)
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- References (continued)
- References (continued)
- Dermatology, 5th Edition (2-Volume Set)