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Extracellular Matrix

Medical Cell Biology

Functions of ECM Provide structural support, regulate intercellular communication, serve as a reservoir for growth factors, prevent the metastasis of some cancer
Components of ECM collagen (most abundant), glycosaminoglycans, proteoglycans, elastic fibres, fibronectin, laminin, basement membrane
Collagen Provides strength and stability to tissue that must change in size and position (tendons, bones, cartilage, skin)
Mutations in coding genes for collagen Achondroplasia, Osteogenesis Imperfecta, Alport Syndrome, Ehlers-Danlos Syndrome
Achondroplasia Skeletal disorder, defect in FGFR-3, dwarfism with short limbs and normal trunk
Osteogenesis Imperfecta Defect in collagen Type I, brittle bone disease, blue sclera
Alport Syndrome Hereditary nephritis, affect renal chochlea, ocular, defect in collagen type IV, progressive renal failure
Ehlers-Danlos syndrome Cutis hyperplastica, defect in collagen type III, affect skin, joints, blood vessels, see easy bruising and hyperelastic skin, joint hyper-mobility, vascular fragility
Glycosminoglycans (GAGs) long carbohydrate chains, highly charged molecule, contain anionic groups (SO4-2), contain hyaluronic acid
Hyaluronic acid Non sulfated GAG, help cartilage resist compression w/o inhibiting flexibility
Proteoglycans sulfated GAGs with polypeptide attached. Ex: aggrecan and syndecan
Aggrecan Resist compression, bind HA to form giant aggregates
Syndecans Are proteoglycans, transmembrane proteins that link cells to ECM
Elastic fibres Network with interwoven collagen to limit stretching and prevent tearing, consists of elastin and fibrillin
Mutation in elastin Narrowing arteries
Mutations in fibrillin Marfan syndrome
Fibronectin Main adhesive molecules that attach cells to other ECM (collagen, haparin, fibrin). Help attach amniotic sac to uterine wall.
Fetal fibronectin Test To determine if the baby is pre-term born. In late pregnancy, if see fibronectin means the early delivery occur
Laminin adhesion protein of basement membrane. Bind other ECM components to basement membrane
Basement Membrane Highly cross-linked layers of ECM anchor all epithelial cells to connective tissue. Provide barrier, compartmentalization/shape and polarity/ signal transduction
Goodpasture Syndrome Ab produced against TYPE IV COLLAGEN in lungs and kidneys cause lung hemorrhagic with nephritis
Fibrins Form blood clots, thrombin cleaves fibrinogen = fibrin
Plasminogen Plasmin (cleaves fibrin)
What happens when Fibrin binds to CAMs Promote angiogenesis
von Willebrand Factor Required for platelet adhesion and maintain normal level of clotting factors VIII. Binds to collagen and to platelets form bridge, aggregation of platelets form and enhance blood clots formation
Rupturing of atherosclerotic plague will cause? Activation of platelets, adhesion to vWF - thrombus formation
Thrombocytopenic Purpura (TPP) Autoimmune/mutations in gene coding for ADAMTS13, clots cannot break down so see patches of redness in the legs
ADAMTS13 Break/cleave vWF multimers, dissolved clots normally in our body
Created by: jsabangan