Carbs V Flashcards
proteoglycans
very large molcules that consist mainly of acidic modified sugars
where does the glycosylation of cell surface proteins occur
in ER and golgi apparatus
functions of ECM
elasticity to cartilage
tensile strength to tendons
cohesion to CT
viscosity to ocular fluids
what does ECM owe it’s mechanical properties to
collagen and proteoglycan
glycosaminoglycans
attached carb side chains
what type of charge do GAGs have
negative
why is the GAG negative charnge important
cations are attracted to it and get trapped in the ECM, giving it gel like properties
what does GAGs considte of
repreating units of amino sugar and acid
how are proteoglycans syntehsized
sugar reidues added to both serine and asparagine resides
sulfate groups added
linked to hyaluronic acid
what does the hyaluronic acid linkage require
linker protein
where are proteoglycans degraded
in lysosomes
what are proteoglycans linked to
long polymers of acids and aminosugars which are sulfating in golgi apparatus after synthesis
what do proteoglycans required for degradation
specialized vacuolar enzymes
mucopolysaccharidosis
defective proteoglycan degrading hydrolase, causing substrate to accumulate in lysosomes
what gets accumulated in hunter syndrome
dermatan sulfate
heparan sulfate
what is the defective enzyme in hunter syndrome
iduronate sulfatase
symtptoms of hunter syndrome
skeletal abnoramlity
intellula disability
what gets accumulated in hurler’scheie
dermatan sulfate
herparan sulfate
what is teh defective enzyme in herler schie
alpha iduronase
what is teh symptoms for herler scheie
skeletal abnoramlity, intellectual disability
what gets accumulated in sanfilippos
heparan sulfate
waht are teh symptoms for sanfilipposns
mild physical defects
severe intellectual diability
for the glycosylation of proteins what does the cell need to synthesize mainly
mannose
galactose
fucose
what is teh general pathway for synthesis of imporatant sugars from glucose
- UDP galactose synthesized
- fructose 6 phophate —> mannose 6 phospahte
- GDP mannose —> GDP fucose
