‼️BIOCHEM-Special Topics Flashcards

1
Q

Examples of acidic sugars

A

Glucuronic acid

Iduronic acid

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2
Q

GAG in mast cells

A

Heparin

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3
Q

All GAGs are attached to proteins to form

A

Proteoglycans

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4
Q

GAG located at sites of calcification in endochondral bone and are also found in cartilage

A

Chondroitin sulfate

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5
Q

GAGs that play a role in corneal transparency

A

Keratan sulfate I and dermatan sulfate

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6
Q

GAG Structural role in sclera

A

Dermatan sulfate

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7
Q

Heparin binds with factors

A

IX and XI

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8
Q

GAG that determines the charge selectiveness of renal glomerulus

A

Heparan sulfate

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9
Q

Synthesis of GAGs occur in which organelles?

A

ER and golgi apparatus

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10
Q

How are GAGs degraded?

A

They are phagocytosed and vesicles fuse with lysosomes where they are degraded

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11
Q

Lysosomal degradationof GAGs are accomplished by

A

Acid hydrolases

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12
Q

Accumulation of GAGs in lysosomes due to deficiency in hydrolases

A

Mucopolysaccharidoses

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13
Q

All mucopolysaccharidoses are autosomal recessive except for

A

Hunter’s syndrome (x-linked recessive)

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14
Q

All mucopolysaccharidoses have mental retardation except

A

Morquio’s syndrome

No CNS involvement

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15
Q

Mucopolysaccharidoseses with deficiency in alpha L iduronidase

A

Hurler’s and Scheie’s syndrome

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16
Q

Type VII mucopolysaccharidoses

Enzyme deficiency?

A

Sly syndrome

Beta glucoronidase

17
Q

Type II mucopolysaccharidoses

Enzyme deficient?

A

Hunter’s syndrome

Iduronate sulfatase

18
Q

Type III mucopolysaccharidoses

A

Sanfilippo syndrome

19
Q

Morquio’s syndrome is deficient in

A

Galactose 6 sulfatase

20
Q

Proteins to which oligosaccharides are covalently attached

A

Glycoproteins

21
Q

Length of carbohydrate chain in gycoprotein is relatively

Vs proteoglycans

A

Short and branched

Proteoglycans: linear and longer

22
Q

Sugar units in glycoproteins vs proteoglycans

A

GP - NO repeating sugar units

PG- WITH repeating sugar units

23
Q

Major classes of glycoproteins

A

O- linked
N-linked
GPI-linked

24
Q

O-linked glycoproteins are bound to which amino acids

A

Serine and threonine

25
Q

N-linked glycoproteins are bound to what amino acid?

A

Asparagine

26
Q

Production of O-linked vs N-linked Glycoprotein

A

O-linked gOlgi apparatus

N-linked eNdoplasmic ret

27
Q

R group

O-linked vs N-linked

A

o- hydroxyl

n- amide

28
Q

Glycosaminoglycans are generally composed of

A

Repeating disaccharide units

Amino sugar
Acidic sugar