Term
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Definition
| this is a non sulfated GAG that is not linked to any proteins |
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Term
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Definition
| most abundant, found in cartilage, tendons, aorta, ligaments, and combine with Hyaluronic Acid |
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Term
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Definition
| only intracellular GAG, found in mast cells of liver, lungs and skin |
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Term
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Definition
| this GAG is found in the basement membrane/cell surfaces |
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Term
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Definition
| heterogenous with galactose; cornea, bone cartilage and combine with chondroitin sulfates |
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Term
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Definition
| these are highly negatively charged GAGS |
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Term
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Definition
| GAG synthesis use what precursor? |
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Term
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Definition
| what pathway do acidic sugars use? |
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Term
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Definition
| D-glucoronic acid and L-Iduronic; how are they related? |
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Term
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Definition
| in this type of autosomal recessive diseases corneal clouding will be present and there is a deficiency with alpha-L-Iduronase |
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Term
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Definition
| in this x linked disease corneal clouding will not be present and there will be an iduronate sulfatase deficiency |
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Term
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Definition
| this syndrome is caused by a deficiency in heparan sulamidase |
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Term
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Definition
| this is caused by a deficiency in NAG (N-Acetylglucosaminidase |
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Term
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Definition
| this is due to a deficiency in GNAT (glucosamine-N-acetyltransferase) |
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Term
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Definition
| this is due to a deficiency in NAG-6-S (N-acetylglucosamine-6-sulfatase) |
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Term
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Definition
| this is due to a beta glucuronidase deficiecny |
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Term
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Definition
| this sugar attachment occurs co translationally |
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Term
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Definition
| this sugar attachment occurs post-trasnslationally |
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Term
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Definition
| this enzyme is abesent in humans and is the reason we need ascorbic acid |
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Term
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Definition
| this enzyme inhibits glycosyl transferases |
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Term
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Definition
carbohydrate-binding proteins
No enzymatic activity
Bind reversibly to mono- and disaccharides with high specificity
are examples of |
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Term
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Definition
What disease is this? increased enzyme serum levels
Defective targeting pathway no P to Man attached incorrect targeting
Characteristics of this disease;
Skeletal abnormalities
Restricted joint movement
Coarse facial features
Severe psychomotor impairment
Death at around 8 years of age
Liver cells normal due to different targeting mechanism to lysosomes |
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Term
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Definition
| this blood type is the o antigen + NAG |
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Term
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Definition
| this blood type is the o antigen + galactosamine |
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Term
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Definition
| this blood type is the o antigen only |
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