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EOM 1 - Test 4 > Hardman - Acylglycerols and Sphingolipids > Flashcards

Hardman - Acylglycerols and Sphingolipids Flashcards

1
Q

Tay Sachs

A

Hexosaminidase A

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2
Q

Fabry’s Disease

A

alpha-galactosidase

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3
Q

Methachromatic Leukodystrophy

A

arylsulfatase A

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4
Q

Krabbe’s Disease

A

Beta-galactosidase

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5
Q

Gaucher’s Disease

A

Beta-glucosidase

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6
Q

Niemann-Pick

A

sphingomyelinase

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7
Q

Farber’s Disease

A

Ceramidase

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8
Q

What is the common intermediate in synthesis of TAGs and phospholipids

A

Phosphatidate

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9
Q

Steps in synthesis of phosphatidate

How many fatty acids does this have?

Which on would likely be saturated?

A
  1. Dihydroxyacetone + Glycerol = L-Glycerol 3-Phosphate
  2. Add 1-FA = Lysophosphatidate
  3. Add 1-FA = Phosphatidate
    - 2 Fatty Acids
    - Usually Carbon #2
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10
Q

From phosphatidate (2-fatty acids + glycerol + phosphate), how do you add a third Fatty Acid?

A
  1. Remove Phosphate (phosphatase)
  2. Add ACTIVATED fatty acid
    - CoA activates
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11
Q

What enzyme in the blood is required to release glycerol from the TAG in blood?

A

Lipoprotein Lipase

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12
Q

What are the steps of glyceroneogenesis?

A

Pyruvate

(Pyruvate Carboxylase)

OAA

(Pep CarboxyKinase)

PEP

I

Dihydroxyacetone Phosphate

(Glycerol 3-phosphate dehydrogenase)

Glycerol 3-phosphate

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13
Q

What does elevated FFA in blood result in?

A
  1. Increase insulin resistance
  2. Muscle can’t handle glucose directly
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14
Q

What is result of glucocorticoids (stress hormones) on the TAG cycle?

A

INHIBITS: PEPCK in Adipose Tissue–shuts down glyceroneogenesis

ACTIVATES: PEPCK in Liver Tissue–increases glyceroneogenesis

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15
Q

How does Thiazolidinediones act on tissues?

What is this a common treatment for?

A

Activates: PEPCK in Adipose Tissues–spins up glyceroneogenesis

Type II Diabetes

- Increase STORAGE of Fatty Acids in Adipose Tissue

  • Reduce Fatty Acid circulation
  • Tissues become more dependent on Glucose
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16
Q

What is the effect of insulin on Carb/Protein metabolism, and Acetyl-CoA?

A

Activates Acetyl-CoA production

Activates Fatty Acid synthesis from Acetyl-CoA

You just ate, insulin high. You want to product Acetyl-CoA to make energy, and use any leftover to begin storage

17
Q

What are four components of phospholipids?

A
  1. Fatty Acid
  2. Glcyerol or Sphingosine Backbone
  3. Phosphate Group
  4. An “Alcohol” (choline, etc)
18
Q

What is the order for synthesis of phospholipids?

A
  1. Synthesis of backbone
  2. Attach Fatty Acids
  3. Add hydrophilic head group through phosphodiester bond (phosphate)
  4. Alter head group with “alcohol”
19
Q

What must be done before addition of final “alcohol” group to phospholipid?

A

Either phosphatidate or alcohol must be activated with CDP

20
Q

What are the three most common “alcohol” moieties?

A

Choline

Glycerol

Inositol

21
Q

Cardiolipin

A

Diphosphatidul Glycerol

Basically two phospholipids with a shared glycerol “alcohol” in the middle

22
Q

How is phosphatidylcholine synthesized?

A

Methylation of phosphatidylethanolamine

23
Q

How is phosphatidylserine synthesized?

A

Base Exchange reaction with phos-choline or phos-ethanolamine

24
Q

What is the structure of sphingosine?

A

Single fatty acid on carbon 3 w/ 2x “open” carbons

  • One amine group on open carbon 2
  • One alcohol group on open carbon 1
25
Q

What are starting products of synthesis of 18C sphinganine?

What is end product (initial starter molecule)?

How is this easily recognized?

A

Starting = Palmitoyl CoA + Serine

End Product = Ceramide

Easily recognized (unique) amide linkage on carbon #2

26
Q

What is the source of the backbone in sphingomyelin?

27
Q

Cerebroside

A

Sphingosine backbone with sugar unit on head–

Glucose or Galactose

Carbon #2 has fatty acid w/Amide Linkage

28
Q

Ceramide + Phosphatidylcholine

A

Sphingomyelin

29
Q

Ceramide + UDP-glucose

A

Cerbroside

30
Q

Cerebroside + Activated Sugars

A

Gangliosides

31
Q

Farber’s has accumulation of?

32
Q

Tay Sachs has an accumulation of?

A

Gangliosides

33
Q

Plasmalogen

A

Ether alkene phospholipid

  • Prevalent in cardiac tissues
  • May protect against ROS
34
Q

Platelet Activating Factor

A

Ether Linked Alkane

Ether glycerolphspholipid

EOM 1 - Test 4 (19 decks)

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