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MCB II Block 2 > Glycosaminoglycans and glycoproteins > Flashcards

Glycosaminoglycans and glycoproteins Flashcards

1
Q

GAGs stand for

A

glycosaminoglycans

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2
Q

what are GAGs

A

modified sugars (glusocamine, iduronic acid, etc). their structure is for their function

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3
Q

proteoglycan aggregates are present in

A

cartilage

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4
Q

GAGs structure describe

A

hyaluronic acid with core protein with proteoglycan monomer

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5
Q

synthesis of GAGs come from

A

intermediates of glycolysis

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6
Q

glutamine to glutamate is it ender or exerognic

A

exergonic

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7
Q

when you are intermediate to polymer you need

A

high energy bond

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8
Q

what is the high energy bond used when you are intermediate to polymer

A

UTP

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9
Q

all amino sugars stem from

A

glucosamine-6-P

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10
Q

glucosamine-6-P originates from

A

fructose-6-P and glutamine

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11
Q

normal sugar metabolism supplies all

A

sugar components

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12
Q

UDP glucuronate is used how

A

to make things that are not soluble in water be able to be used in water and be in water.

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13
Q

what is mediating connection b/w polymer and the protein in carbohydrate chain

A

xylose, galactose, galactose

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14
Q

b/w the protein and the sugar there is what bond

A

O-linked glycosidic bond

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15
Q

the addition of sugars is via activated

A

nucleotide donors

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16
Q

GAGs are located where

A

extracllular

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17
Q

to degrade GAGs what needs to appen

A

they must first be engulfed by invagination of cell membrane, forming vesicle inside of cell

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18
Q

where are GAGs degraded

A

lysosomes

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19
Q

when we age, we start to lose cartilage and when we lost it is because of

A

degradation of cartilage causes inflammation and causes pain
doctors recommend vitamins: chondroitin sulfate in the hope the chondrocytes will produce more cattilage to replace what is being degraded

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20
Q

what do doctors recommend to old people in pain b/c they are losing cartilage

A

taking vitamins to help their body make more cartilage

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21
Q

what is another name for glucosaminoglycans

A

mucopolysaccharides

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22
Q

MPS is the same as

A

GAGs

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23
Q

explain hereditary disease of mucopolysaccharides

A

The mucopolysaccharidoses are hereditary diseases (approximately 1:25,000 live births) caused by a deficiency of any one of the lysosomalhydrolasesnormally involved in the degradation of heparan sulfate and/or dermatan sulfate

They are progressive disorders characterized by lysosomal accumulation of GAG in various tissues, causing a range of symptoms, such as skeletal and ECM deformities, and intellectual disability.

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24
Q

where do MPS diseases take place

A

lysosome

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25
Q

GP stands for

A

glycoprotein

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26
Q

GP

A

mostly protein, a little bit carbohydrate

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27
Q

in GPs proteins and oligosaccharides are attached how

A

covalently

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28
Q

virtually all of our secreted globular proteins are

A

glycoproteins

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29
Q

what is function of GPs

A

lots! cell markers, antigenicity, receptors, attachment sites, lubrication of body passages & vasculature, nutrient absorption (eg: Vit B12 – requires intrinsic factor from stomach)

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30
Q

what is linkage of GAGs?

A

glycosidic linkage

serine linkage

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31
Q

what is linkage of glycoproteins

A

N-link

O linked

32
Q

what are the two glycosidic bonds a glycoprotein can have

A

N or O linked (can also have both)

33
Q

describe N-linked

A

glycosidic bond b/w anomeric carbon in carbohydrate and aspragaine

34
Q

look at pic of glycoprotein linkage

A

pg 21

35
Q

synthesis of o linked glycosides is similar to that of

A

GAGs

36
Q

draw out the assembly of N linked glycoproteins

A

pg 23

37
Q

where is N linked glycoproteins happenign

A

ER (this is unusual)

38
Q

mannose has to be phosphorylated to produce

A

mannose 6 phosphate

39
Q

when mannose isn’t phosphorylated what happens to it

A

it is secreted

40
Q

I cell disease is due to absent

A

lysosomal glycoprotein hydrolases

41
Q

show the lysosomal hydrolase and he two steps in the mannose phosphorylation pg 24

A

pg 24

42
Q

what are classic features of I cell disease

A

skeletal abnormalities & joint
movement; severe psychomotor
impairment; death by 8 yrs common

43
Q

what is on the surface of blood for ABO system

A

glycoprotein

44
Q

UDP glucuronate is a product of

A

UDP glucose

45
Q

what will UDP glucuronate be used to make

A

lots of things!
especially GAGs (iduronate, UDP-xylose)
steroids, drugs, bilirubin
proteoglycans

46
Q

when glucuronate is attached what does it do

A

negative charge,
raises solubility,
enhanced water-holding

47
Q

where is the core protein synthesized

A

by ribosomes on RER

48
Q

after the core protein is synthesized in ER, what happens

A

it is glycosylated by membrane membrane-bound glycosyltransfererases located in the Golgi

49
Q

defect in sulfation of GAGs result in

A

disorder: chondrodystrophies (affects proper development and maint. of skeletal system)

50
Q

when does sulfation of a GAG occur?

A

after the particular monosaccharide to be sulfated has been incorporated into the growing carbohydrate chain

51
Q

chondroitin sulfate is a

A

sulfated GAG

52
Q

sulfation reaction is catalysed by

A

sulfotransferases

53
Q

what is the source of sulfate for sulfating GAGs

A

PAPs

54
Q

what does Chondrodystrophies affect

A

proper development and maint. of skeletal system

55
Q

hereditary disorder of GAG degradation:

A

Mucopolysaccharidoses

56
Q

where is the enzyme deficiency in Mucopolysaccharidoses

A

deficiency of a lysosomal hydrolase

57
Q

name three examples of Mucopolysaccharidoses

A

hunter
hurler’s
sanfilppo

58
Q

O-linked oligosaccharides can have what structure

A

linear or branched

59
Q

draw the A and B blood antigen

A

pg 26

60
Q

what forms the H antigen, the substructure of A and B blood groups

A

fructose linked to galactose

61
Q

MPS stands for

A

Mucopolysaccharidosis

62
Q

what is the most severe of all MPSs

A

Hurler’s

63
Q

corneal clouding, mental retardation, dwarfing, coronary artery blockage, early death
is what disease

A

Hurlers

64
Q

where is the specific enzyme defect in Hurler’s

A

Iduronidase

65
Q

hurler’s disease and it’s defect in iduronidase ultimately leads to what

A

accumulation of non-degradable GAGs

66
Q

where are a lot of GAGs found

A

in joins

67
Q

what do GAGs function as in joints

A

shock absorber

when compressed, water goes out, then water is let back in when compression stops and reforms its shape.

68
Q

what is the charge of GAGs and how does this affect its relationship with water

A

It is negative, it attracts water

69
Q

draw the general linkage b/w core protein and rest of GAG

A

pg 6

70
Q

what is a triheoxide

A

galactose-galactose-xilose

71
Q

what is biological significance of dermatan sulfate

A

skin, blood vessels, heart valves

72
Q

what is the biological significance of chondroitin 4 and 6 sulfates

A

in cartilage

they bind collagen and hold fibers a tight, strong network

73
Q

what is the biological significance of keratan sulfates

A

KS I in cornea

KS II in loose CT

74
Q

what is the biological significance of heparin

A

anticoagulant

75
Q

what is the biological significance of hyaluronic acid

A

synovial fluid of joints, vitreous humor of eye, umbilical cord, loose CT, cartilage

76
Q

what is the biological significance of heparan sulfate

A

extracellular GAG found in basement membrane

MCB II Block 2 (10 decks)

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