ECM Flashcards
basic elements of the ECM
ground substance
fibers
granulation tissue
syndecans
proteoglycan
core protein mcl is embedded in the cell membrane as a transmembrane protein
serves as a receptor for fibroblast growth factor
perlecans
proteoglycan in basement membrane
functions: cell adhesion, proliferation, differentiation, glomerular filtration, development, and growth factor binding
enhanced in some tumors
adhesive glycoproteins have 3 binding sites
- integrins
- collagen fibers
- proteoglycans
fibronectin
binds to inegrins, collage, heparin, heparan sulfate, and hyaluronic acid
-located in connective tissue, blood plasma, and embryonic tissue
laminin
binds to integrins, heparan sulfate, collagen IV, and entactin
located in basal lamina
entactin
binds to laminin and type IV collagen
located in basal lamina
tenascin
binds to syndecans and fibronectin
located in embryonic tissue
chondronectin
binds to collagen II, chondritin sulfates, hyaluronic acid, and integrins of chrondrocytes
located in cartilage
osteonectin
binds to collagen I, proteoglycans, integrins of bone cells
located in bone
collagen I
tissue: dermis, tendon, bone, dentin, cementum, fibrocartilage, organ capsules
cells: fibroblasts, ondontoblasts, cementoblasts, osteoblasts, chrondroblasts
function- resistance to tension, fibril forming leading to fibers
collagen II
tissue: hyaline and elastic cartilage
cells: chrondroblasts
function: resistance to pressure, fibril forming
collagen III
tissue: spleen, liver, lymph nodes, smooth muscle, skin, lung
cells: smooth muscle cells, fibroblasts, reticular cells
function: maintains structure, fibril forming to fibers
collagen IV
tissue: basal lamina
cells: endothelial cells, epithelial cells, schwann cells, smooth muscle cells
function: support delicate structures, network forming
collagen VII
tissue: dermis
cells: epidermal cells
function: anchors lamina densa to the lamina reticularis, anchoring collagen
ehlers danlos IV
COL3A1 mutation in type III collagen
varicose veins, aortic rupture, intestinal rupture
ehlers danlos VI
defective hydroxylation of lysine, destabilizing collagen strength
hyperelasticity of skin, rupture eyeball
ehlers danlos VII
COL1A1 & COL1A2 mutations encoding collagen I
joint dislocation and hypermobility of joints
scurvy
decreased hydroxylation of proline, deficiency in vit c
gum ulceration and hemorrhages
osteogenesis imperfecta
COL1A1 mutation reduced synthesis collagen I
spontaneous fractures and cardiac insufficiency
strickler syndrome
COL2A1 mutation for collagen II
myopia, hypoplasia of mandible, arthritis
marfan syndrome
mutation on fibrillin 1 gene on chromosome 15
aortic aneurysm, myopia, detached lens, skeletal defects
