Glycoproteins and Proteoglycans X Flashcards

1
Q

Glycoproteins - def

**

A

proteins that covalently attach carbohydrate chains EXCEPT polymers of repeating units
Carb chains - short, unbranched
varying amounts and concentrations of carb

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2
Q

Proteoglycans - def

**

A

polysaccahrides attached to core protein

can have as much as 95% carb

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3
Q

Carbohydrate - types

A
Simple sugars
Modified suagars (like adding amino groups)
Polysaccharides
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4
Q

Simple Sugars

A

monosaccharides

i.e. glucose, mannose, galactose

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5
Q

Modified sugars

A

principal = hexosamines (N added to C2)

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6
Q

Polymers

A
putting sugars linked with glycosidic bonds
, Di-, Tri-, Oligo- and Poly-
i.e. maltose, lactose
put together by glycosyltransferases
taken apart by glycosidades
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7
Q

Glycosylation

A

attaching carbs
not template driven
Can use N or O linkages

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8
Q

known N and O linkages

**

A

N-glycosyl to asparagine
O glycosyl to serine or theorine
O glycosyl to hydroxylysine

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9
Q

Glycolysation - functional impact

A

Increase protein solubility
guide protein folding
provide some protections from proteolytic degradation to the polypeptide backbone
+other regulation events

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10
Q

Glycosylation - critical for

A

Naturally occurring proteins in higher concentrations

Biopharmaceuticals

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11
Q

Blood Group Substances (naturally occurring protein)

A

Different carbohydrates recognized for blood group definition

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12
Q

aberrant glycosylation

A

Can affect whether substrate get recognized by enzyme

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13
Q

aberrant glycosylation patterns of proeints

A

can be analyzed and validates as potential biomarkers of disease

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14
Q

Biopharm - i.e.

A

Erythropoitein, Myozyme

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15
Q

degradation of glycoprotiens - pthwy

A

sugars removed from non-reducing end, by lysosomes.

Opposite of construction

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16
Q

Enzyme deficiency results in progressive accumulation of aspartylglucosamine in tissues, spinal fluid and urine

A

KNOW

17
Q

I cell disease - a.k.a, def

***

A

Mucolipidosis, Lysosomal Storage disorder with UDP-N-acetylglucosamine-I-phosphotransferase deficiency
*enzyme that puts sugar on glycoproteins

18
Q

Mucolipidosis - physcial manifesations

***

A

corenal clouding
short stature
umbilical hernias.

19
Q

proteoglycans

A

carbs with protein core
high anionic charge - basophilic (blue)
disaccharide repeating unit + sulfate (negative charge)

20
Q

GAG - a.k.a, types

**

A

**glycosaminoglycans
Chonroitin Sulfate (most commoon, i.e. aggrecan)
Dermatan Sulfate
Hepran Sulfate
Keretan Sulfate
Heparin (huge clusters of negative charge = coagulant)
Hyaluronic acid

21
Q

Disaccharide repeating unit

A

uronic acid and amino sugar

negative charge

22
Q

Aggrecan loss from the cartilage ECM is an early and sustained feature of OA

A

KNOW

23
Q

Proteoglycans - role

**

A

support/connective tissue
mechanical
diffusion

24
Q

Proteoglycans - location

A

intracellular compartment, cell surface or in ECM

25
Q

Classes of Proteoglycans

***

A

Large aggregating proteoglycans *(i.e.AGGRECAN)
small leucine-rich proteoglycans (SLRP)
basement membrane proteoglycans

26
Q

Hyaluronate/Hyaluronic Acid - def, fxn

A

GAG - not proteoglycan
co ploymer of Nacetyleglucoamine and Glucuronic Acid
shock absorgber, lubricant

27
Q

Heparin

A

unlike other GAG’s, intracelluar component of mast cell

used to contract thrombosis(clot)

28
Q

Lubricin

A

Major lube in joint - boundary lubricant

29
Q

degradation of proteoglycans

A

one at a time

30
Q

Mucopolysaccharidoses - def

***

A

genetic disorder of excessive accumulation and excretion of the oligosaccharides of proteoglycans

31
Q

Mucopolysaccharidoses - i.e.

***

A

Hunter

Hurler + Scheie

32
Q

Hunter - Accumulated products, Deficient enzyme, cleaves

***

A

Accumulated products - heparin sulfate, dermatan sulfate
Deficient enzyme - Iduronate sufatase
*cleaves sulfate from hylauronic acid

33
Q

Hurlers + Scheie - Accumulated products, Deficient enzyme, cleaves
***

A

Accumulated products - heparin sulfate, dermatan sulfate
Deficient enzyme - alpha-L-Iduronidase
*Cleaves hylauronic acid