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Biochemistry Pulmonology > Glycosphingolipids (GSLs) > Flashcards

Glycosphingolipids (GSLs) Flashcards

1
Q

Glycosphingolipids (GSLs)

A

Glycogonjugates that are major parts of biological membranes

Each contain a hydrophilic sugar chain linked to a hydrophobic ceramide

The ceramide anchors it to the membrane

Act as signaling molecules to regulate differentiation, proliferation, and apoptosis

Neutral and acidic

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2
Q

Ceramides

A

Hydrophobic sugar chain in the GSLs

= a sphigosine backbone acylated by a FA

In skin, act as a water permeability barrier

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3
Q

Gangliosides

A

Major acidic acidic GSLs

= sialic acid-containing GSLs

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4
Q

Galactosylceramide (-cerebroside)

A

Neutral GSL

Galactose beta linked to ceramide

Found mainly in CNS

Associated with Krabbe Disease

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5
Q

Glucosylceramide (-cerebroside)

A

neutral GSL

Glucose beta linked to ceramide

Found in visceral organs

Associated with Gaucher disease

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6
Q

Lactosylceramide

A

GalB1–>4GlcB—>Cer

Notice: glycolipid = lactose

Neutral

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7
Q

Globotriaosylceramide (Gb3)

A

GalA1–>5GalB1–>4GlcB—>Cer

**adding another Galactose to lactosylceramide

Found in visceral organs

Associated with Fabry Disease

Neutral

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8
Q

Globotetraosylceramide (Gb4)

A

GalNAcB1–>3GalA1–>4GalB1–>4GlcB—>Cer

**adding GalNAc to Gb3

Visceral organs

Neutral

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9
Q

Catabolism of Gb4

A

Terminal B-linked GalNAc is removed —> yields Gb3

Beta-hexosaminidase A and B

Blocked in Type O Tay Sachs

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10
Q

Beta-hexosaminidase A & B (HexA and HexB)

A

both can cleave both beta linked GlcNAc and GalNAc structures

Hex A = 1 alpha and 1 beta

Hex B = 2 betas

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11
Q

Catabolism of Gb3

A

A1,4 terminal galactose is removed

Enzyme: alpha-galactosidase A

PRODUCT: lactosylceramide

Associated with Fabry Disease

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12
Q

Catabolism of lactosylceramide

A

Terminal beta1,4 galactose removed

ENZYME: beta-galactosidase

PRODUCT: glucosylceramide

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13
Q

Catabolism of glucosylceramide (GlcCer)

A

ENZYME: beta-glucosidase

PRODUCT(S): glucose and ceramide

Associated with Gaucher Disease

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14
Q

Catabolism of galactosylceramide (GalCer)

A

ENZYME: galactocerebrosidase (a beta-galactosidase)

PRODUCTS: galactose and ceramide

Associated with Krabbe disease

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15
Q

What sialic acid do gangliosides contain (acidic GSLs)

A

N-acettylneuraminic Acid (NeuAc)

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16
Q

The 3 acidic GSLs (gangliosides)

A

GM3 = shortest sugar chain

GM2

GM1

17
Q

GM3

A

NeuAc attached to the terminal Gal of the neutral GSL (lactosylceramide)

Major ganglioside in visceral organs

18
Q

GM2

A

Attaching an GalNAc to the galactose of the GM3

Beta-1,4

Associated with Tay-Sachs Disease

19
Q

GM1

A

Attaching Gal to the termainl GalNAc of the GM2

Beta-1,3 linkage

Major ganglioside of the CNS

Receptor of the cholera toxin

20
Q

Nomenclature of gangliosides

A

GM#

G = ‘ganglioside’

M# = number of sugar cahins (5-n)

—> n= number of sugar units in the main chain

Ex: GM1 because it has 4 sugars in the chain

21
Q

Catabolism of GM1

A

Terminal Galactose (beta1,3)

Enzyme: beta-galactosidase

Associated with GM1-gangliosidosis (or generalized gangliosidosis)

22
Q

Catabolism of GM2

A

Terminal GalNAc is hydrolyzed (B1,4)

Enzyme: beta-hexoaminidase A (Hex A)

co-factor: GM2-activator

…now have GM3

Associated with Tay Sachs

23
Q

Catabolism of GM3

A

terminal NeuAc (alpha2,3)

Enzyme: sialidase (neuraminidase)

…now have lactosylceramide…wich can be converted to glucosylceramide with a beta-galactosidase

24
Q

Basic unit of the all antigens on RBCs

A

GalB1–>4GlcNAcB1–>RBC

25
Q

H-antigen

A

Blood group O

L-fucose linked to terminal Gal (alpha1,2)

H-determinant = this dissaccharide

26
Q

A-antigen (RBC)

A

If GalNAc is attached to the H-antigen

Alpha 1,3

A-determinant = this tri-saccharide

27
Q

B-antigen (RBC)

A

If Gal (a1,3) attached to H-antigen

B-determinant

Biochemistry Pulmonology (12 decks)

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