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Extracellular matrix
Tufts D17 Exam IV
38
Biochemistry
Professional
11/13/2013

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Term
3 main roles of ECM
Definition
Mechanical, protective, organization
Term
What is the second step of extracellular protein formation?
Definition
Hydroxylation which occurs commonly on praline and hydroxylysine
Term
What enzyme causes Hydroxylation of praline and lysine?
Definition
prolyl or Lysol hydroxylase. The cofactor is vitamin C. Scurvy.
Term
What is step 3?
Definition
Glycosylation. Hydroxylysine ad asparagine are glycosolated (galactose/glucose added).
Term
Step 4?
Definition
Disulfide bond formation at the C terminus align the three alpha chains before the triple helix
Term
Step 5
Definition
The central protons of the chains zipper from C- to Ne-terminus to form the triple helix.
Term
What is immature collagen called?
Definition
procallogen.
Term
What are the n- and c- terminals called
/
Definition
propeptides
Term
Step 8?
Definition
tropocollogen molecules spontaneously assemble into a fibrin with holes.
Term
What stabilizes the collagen maturation? Step 9?
Definition
Cross inks between aldehyde. (lysyl oxidase)
Term
What is Ehlers-Danlos?
Definition
Stretchy skin, joints. 1. Deficient collagen fibrin formation. (defective peptidase) 2. Deficient crosslinking in collegian.(lysil oxidase)
Term
Where is elastin found?
Definition
Lungs, skin, blood vessels
Term
What elastin core is necessary for elastin?
Definition
fibrillin. Crucial for integrity of elastic fibers.
Term
What is Marian syndrome?
Definition
Elastic fibers are weak, tall, crowded teeth, chest abnormalities, anyuerisns
Term
GAGS?
Definition
Long unbranched polysaccharides consisting of Disaccharides.
Term
GAG charge?
Definition
Negative due to sulfate
Term
Gags are hydrophilic. What happens in water?
Definition
h20 sucked into matrix and a gel forms.
Term
What is an example of gags:
Definition
Articular cartilage
Term
proteoglycans are found where?
Definition
Cartilage.
Term
What disease is associated with proteoglycans
Definition
Arthritis.
Term
What is wrong with PROTEOGLYCANS in osteoarthritis?
Definition
Decreased GAG synthesis by chondroblasts. Decreases PROTEOGLYCANS made from gags.
Term
What type of collagen is in the bone?
Definition
Type 1
Term
whatcallogen types in dentin and cementum?
Definition
Type 1 and 3
Term
What protein is in enamel
Definition
amelogenin
Term
What protein is in dentin
Definition
Dentin sialophosphoprotein DSPP
Term
Amelogenis imperfecta
Definition
Mutations in amelogenin gene which plays role in enamel formation during development.Causes enamel is abnormally thing. Yellow and brown. Enamel is easily damaged and sensitive to temp
Term
TYPE I dgi
Definition
Patients have mutations in collagen I and affects dentin mineralization. Bones are worse.
Term
Type II DGI
Definition
It is the most common dentin inherited disease (1:8000) patients have mutations in non-collagen protein dentin sialophosprotein. Causes abnormal dentin matrix mineralization and discoloration
Term
ECM is subject to turnover
Definition
Not only synthesized but degraded in a controlled fashion. By matrix metallproteases.
Term
What protease is responsible for enamel turnover?
Definition
enamelysin is important for proteolytic processing of extracellular matrix proteins.
Term
What does excessive MMP activity lead to?
Definition
Collagen breakdown of gingiv and bone destruction in periodontitis. Treated by periostat. Doxycylcine inhibits MMP activity
Term
Excessive MMP activity in articular cartilidge caueses
Definition
Osteoarthritis. Excessive degradation of collagen II and PROTEOGLYCANS such as aggrecan by excessive MMP.
Term
Extracellular matrix proteins are associated with
Definition
Cancer.
Term
Classes of molecules in ECM?
Definition
Fibrous proteins (colognes) 2. Polysaccharide chains 3. Proteins with glycosaminoglycans 4. Inorganic crystals.
Term
What is a fibrous protein and most abundant in animal tissues?
Definition
Collagen.
Term
Collagen are made up of what type of sequence?
Definition
Alpha chains mostly glycine.
Term
whatisthe difference between alpha helix and alpha chain?
Definition
3 alpha chains are twisted together into a right handed super helix
Term
What disease is caused by glycine mutations?
Definition
osteogensis imperfecta
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