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Mucopolysaccharidoses
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In what 2 diseases does heparan and dermatan sulfate accumulate?
Hurler's
Hunters
What enzyme is deficient in Hurlers disease?
alpha L iduronidase
What enzyme is deficient in Hunter's syndrome?
Iduronate sulfatase
Which enzyme is deficient in I cell disease?
GlcNac phosphotransferase
are glycoproteins or proteoglycans branched?
glycoproteins branched
proteoglycans --unbranched
Enzymes destined for lysosomes are tagged how?
Mannose 6 phosphate.
Mannose in ER
Phosphylation in Golgi Aparatus
O linked glycosidic bonds use what a.a?
serine, threonine, hydroxylysine
N linked glycosidic bonds use what a.a?
Asparagine
What is the major feature of complex class of oligosaccharides linked to a protein in glycoproteins?
Sialic Acid
O linked carbs are attached in glycoproteins are attached where?
GA
N linked carbs are attached where?
What is needed?
ER
Dolichol phosphate
In glycoproteins the following consensus sequence is attached to a protein: Asn-x-ser/Threonine
X can be any a.a except?
Proline
NANA(sialic acid) is incorporated into a glycoprotein using what high energy molecule
CMP-NANA
In O glycosidic linked carbs what sugar is used
galactose
In N linked glycosidic linked carbs, what sugar is used?
glucose
Which is more common, N or O linked?
N linked
Which one of the glucosaminoglycans is not sulfated and covalently linked to proteins?
Hyaluronic acid
What the function of Hyaluronic acid?
shock absorber
lubricant
found in synovial fluid, vitreous humor, extracellular matrix
Which one of the GAGs does not have uronic acid?
Keratan sulfate
Where is Dermatan sulfate found?
Skin
Blood vessels
Heart Valves
Chondroitin sulfates are found where?
cartilage, tendons
What is the only intracellular GAG
Heparin
What is the trisaccharide linkage in proteoglycans?
gal-gal-xylose-serine
Why are the core proteins of proteoglycans rich in serine and threonine residues??
So that O linkages to carbs can occur
How are proteoglycan monomers held onto Hyaluronic acid?
ionic interactions
What is the source of sulfate for proteoglycans?
paps
L gulonate to ascorbate requires what acid that is absent in humans?
L gulono lactone oxidase
Sulfation and acetylation of proteoglycans occurs when?
after monosaccharides have been incorporated
What property of GAG explains their reaction?
they are negatively charged
What is the repeating disaccharide in a GAG?
Amino sugar-acidic sugar
Proteoglycans bind to what GAG?
Hyularonic acid
Which gag does not have Uronic acid?
Keratan sulfate
What takes place of uronic acid in Keratan sulfate?
Galactose
Are GAGs hetero or homo polysaccharides?
heteropolysaccharides
What are the repeating disaccharides units in GAGs?
D-gluco or galactosamine and D- Gluco or galactoronic acid(Uronic acid)
Which is the GAG that has L sugars and and a variety of other sugars such as NANA?
Keratan sulfate
What makes up the trihexoside linker protein?
Gal-Gal-Xylose-serine
In I cell disease what is not phosphorylated?
Mannose viA THE deficient enzyme GlcNac phosphotransferase
What is the relation between insulin and potassium?
Insulin sequesters potassium in cells
How is Hyperkalemia treated?
Insulin
glucose
What leads to polyphagia in DM?
Lack of insulin prevents entry of glucose into satiety center
lack of entry of glucose disinhibits hunger center
hunger amongst plenty
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Author
pszurnicki
ID
65627
Card Set
Mucopolysaccharidoses
Description
Mucopolysaccharidoses
Updated
2011-02-12T05:34:40Z
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