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Extracellular Matrix
Medical Cell Biology
| Question | Answer |
|---|---|
| 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
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