Term
| What do collagens do for the ECM? |
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Definition
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Term
| What does ECM stand for even? |
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Definition
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Term
| What is the structure of collagen? |
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Definition
| You get these helices that form into fibrils which form into a collagen fiber with multiple fibrils in it. |
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Term
| Are there many different types of collagens? |
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Definition
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Term
| How is collagen biosynthesized? |
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Definition
| It is synthesized as procollagen. The pro gets taken off by procollagen proteases in the ECM. This prevents intracellular aggregation. It is made in the rough ER and then undergoes modifications and is shipped to ECM by the golgi. |
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Term
| How might collagen be attached to cells? |
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Definition
| via fibronectin and integrins |
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Term
| What is the basal lamina? |
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Definition
| It is the part of the ECM secreted by epithelial cells, and it sits right below them. |
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Term
| What is a disease commonly associated with collagen breakdown? |
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Definition
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Term
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Definition
| It is another type of protein in the ECM. It is elastic and provides the ECM with flexibility/resilience. |
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Term
| What is the structure of elastin? |
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Definition
| Cross-linked fibers to form a network like rubber band |
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Term
| What do glycosaminoglycans do? |
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Definition
| They are extremely hydophilic, so they attract water molecules into the ECM. Also, they like to attract ions, so you get more water by osmotic forces. |
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Term
| What charge to GAG's have? |
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Definition
| negative charged polysaccharides |
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Term
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Definition
| They are proteins with glycosaminoglycans attached. |
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Term
| What do proteoglycans do? |
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Definition
| They are the fibrous structural proteins of the ECM. |
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Term
| What are the most important adhesive glycoproteins? |
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Definition
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Term
| Where is laminin found and what does it do? |
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Definition
| It is in the basal lamina and adheres proteoglycans to collagen. |
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Term
| What is fibronectin/what does it do? |
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Definition
| It is found in connective tissue. In the ECM. It binds collagen to proteoglycan. |
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Term
| What mutation happens in Marfan's syndrome? |
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Definition
| you have a mutation on the Fibrillin 1 gene. It is autosomal dominant. So, 50% of children born from Marfan's parents will get it. |
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Term
| What are clinical manifestations of Marfan's? |
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Definition
| You get aortic dilation (and sometimes rupture/dissection). You also get valvular disease (particularly mitral). Also retinal dislocations, joint laxity. Long limbs. |
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Term
| Explain the deal with TGF-B and marfan's. |
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Definition
| TFG-B usually binds fibrillin 1 to make it inactive. It can't bind fibrillin 1 when fibrillin 1 is defective. So, you get hyperactivation of TGF-B. This causes hypertension, aortic root aneurysm, and mitral valve prolapse BECAUSE angiotensin II gets hyperactivated (downstream effector of TFG-B). |
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