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What is the Structure of a Glycosaminoglycan?
Made of repeating disaccharid units containing An acidic sugar (glucuronic acid) then an amino sugar( N-Acetylglucosamine) (Amino group on 2nd Carbon)
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What are three Acidic sugars?
What are 3 amino sugars?
- Glucosamine, D-Glucuronic acid, and Iduronic Acid
- N0Acetylglucosamine, Muramic acid, and N-acetylmuramic acid
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Facts about Hyluronic acid
- No sulfate groups
- No covalent bonds
- Located everywhere
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What structures make up the linkage region of the GAGs?
O-linked Xylose, and 2 galactose
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What holds the Preteoglycans to the Hyluronic Core protein?
Ionic bonds between the hyluronic core and the preteoglycans core. Stabilized by linkage preteins.
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What adds Sulfate to the Carbohydrate chains of GAGs?
PAPS ( 3 phosphoadenosine 5 phosphosulfate )
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Where does degradation in glycosaminoglycans occur?
In the lysosomes.
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What are the different types of Mucopolysaccharidoses and their mutations?
- Hurlers: Alpha L iduronidase (coroneal clouding)
- Hunters: Iduronate Sulfatase (X linked)
- Slys- B-glucuronidase (dermatin and heparan sulfate)
- Sanfilippos-
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Where is dolicol needed?
in the addition of an N linkage
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In the exception or O linkage in Hydroxylysine in collagen, what is needed?
- it is between Galactose ( or glucose) and the OH of the hydroylysine
- It needs Vitamin C
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When to used N-linked oligosaccharides?
- High mannose content
- Complex oligosaccharides
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What are he activator for mannose, fucose and NANA?
GDP,GDP and CMP
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Where are Fucose and NANA located on the Glycoprotein?
At the terminal end.
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I-Cell.
A inability to phorphorylate mannose in the glucose. causes accumulation of things to be degraded.
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Degradation of Glycoproteins
Remember: Last attached is the first removed.
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