-
glycosly transferase is the enzyme that
enlongates the glycoproteins that are attached to the membrane of the golgi
-
proteins that will be exported, incorporated into the membrane or going to lysosome will contain what
a hydrophobic signal sequence on the N-terminal
this acts to target the protein
-
I-cell disease (inclusion body disease) is caused by a deficiency of__________, an enzyme normally found in the cis-Golgi
N-acetylglucosamine phosphotransferase
-
What are the three representative sugars found in GAGs?
- glucosamine
- D-glucuronic acid
- L-Iduronic acid
-
what are the 2 adhesive proteins found in the ECM
laminin, fibronectins
-
Most of the GAGs are sulfonated but one is not...which is this one?
Hyaluronic acid
-
what is the most abundant GAG in the body?
Chondroitin 4 and 6 sulfates
-
found primarily in the skin, but also in blood vessels and heard valves
Dermatan sulfate
-
found primarily in the corner of eye, playing a role in corneal transparency
keratan sulfates
-
found in cartilage, tendons, ligaments, aorta and other part of large aggregates
chondroitin-4 and 6 sulfates
-
heparin binds to _____ and enhances its activity as an anticoagulant (inhibition of thrombin)
anti-thrombin III
-
anti-throbin III is a ______ inhibitor
protease
-
True/False: hylauronic acid is covalently bound to proteins
FALSE
bound but NOT covalently
-
difference between heparin and heparan
heparin is found intracellularly
heparan is a component of cell surfaces
-
proteoglycans are proteins that are covalently linked to ______
GAGs
-
what is the most common linkage between the
core protein and the GAG
O-glycosidic bond between the xylose on the trihexoside (Gal-Gal-Xyl) and a serine residue in the protein
-
The core proteins of the proteoglycans are synthesized in the ____ and how do you know this?
ER
because they are exported to the outside of the cell
-
where does chain elongation, chain termination, and other modifications (additon of sulfates and epimerization of sugars) occur?
in the Golgi
-
what is called when the proteoglycan (core proteins linked to GAG’s) units interact through ionic association
formation of proteoglycan aggregates.
-
the proteoglycan units in the aggregates are associated through ionic interaction (non covalent) with_____ ____
hylauronic acid
-
where does GAG breakdown occur?
lysosome
-
what two enzymes are required in the breakdown of GAGs
endoglycosidases and exoglycosidases
-
Mutations associated with the lysosomal hydrolases are known as
mucopolysaccharidoses
-
the identification of the ____ _____ can indicate which specific enzyme is deficient or absent in a mucopolysaccharidoses
terminal sugar
-
______ is secreted by some bacteria.
This enzyme helps the bacteria spread by enzymatically
Hyaluronidase
loosening the connective tissue matrix.
-
Hyaluronidase has also been used to help promote the absorption and dispersion of what?
Drugs
loosen the CT matrix so drugs can get in
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