Extracellular matrix Flashcards
What is the extracellular matrix
. Complex network of proteins and carbohydrates filling spaces between cells
. Comprises both fibrillar and non fibrillar components
List 4 key functions of the extracellular matrix
.Provides physical support
.Determines the mechanical and physiochemical properties of tissues
.Influences the growth, adhesion and differentiation status of the cells and tissues with which it interacts
.Essential for development, tissue function and organogenesis.
Gene mutations affect matrix proteins. Alport syndrome affects… which is found in the…
Type IV Collagen, basal lamnia
Gene mutations affect matrix proteins. Marfan’s syndrome affects… which is a ….
Fibrillin 1, collagen
Gene mutations affect matrix proteins. Epidermolysis Bullosa affects …
Laminin 5 ( in all 3 chains)
a defect in Laminin 2 is caused by what disorder?
Congenital Muscular Dystrophy
osteogenesis imperfecta affects…
Type 1 collagen
Type 1 collagen is affected by what disorder?
osteogenesis imperfecta
Gene mutations affect ECM catabolism. a.k.a mucopolysaccharidoses (MPS) inability to degrade…
GAGs
Name two disorders that affect ECM metabolism
Hurler’s syndrome, L-alpha-iduronidase
Name three fibrotic disorders due to excessive ECM deposition
Liver fibrosis-cirrhosis
Kidney fibrosis- diabetic nephropathy
Lung fibrosis- idiopathic pulmonary fibrosis (IPF)
Name a disorder due to excessive LOSS of ECM
osteoarthritis
identify the components of connective tissue
Collagens: Type I, II, III (fibrillar) Type IV (basement membrane)
Multi-adhesive glycoproteins
Fibronectin, Fibrinogen
Laminins (basement membrane)
Proteoglycans
Aggrecan, Versican, Decorin
Perlecan (basement membrane)
what are the properties of bone
hard and dense
2 properties of cartilage
resilient+shock absorbing
