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