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Biochemistry Block 3 > Complex Lipids And Disorders > Flashcards

Complex Lipids And Disorders Flashcards

1
Q

Name 1 storage/simple lipid

Name 2 membrane/complex lipids

A

Triacylglycerols

Phospholipids and glycolipids

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2
Q

Two sub sections of phospholipids

What is their difference

A

Glycerophospholipids and sphingolipids

One has glycerol backbone and other has sphingosine backbone

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3
Q

Sub section of glycolipids

A

Sphingolipids

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4
Q

What is difference between phospholipid sphingolipid and glycolipid sphingolipid?

A

Glycolipid one has a sugar attached; other one has PO4 and secondary alcohol (choline) attached

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5
Q
  1. Main role of phospholipids

2. Phospholipids serve as a component of what 3 things

A
  1. Important in cell membrane for signaling and anchoring proteins etc.
  2. Lipoprotein particles, pulmonary surfactants and bile
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6
Q

What is the most important precursor for glycerophospholipids?

A

Phosphatidic acid (PA)

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7
Q

What makes phospholipids different from each other?

A

All have PA, difference is in their secondary alcohol group

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8
Q

*What is necessary in order to synthesize phospholipids

What is its function

A

CDP

Activates either the alcohol or DAG component - activates the head group (similar to how UDP was activator of glucose)

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9
Q

Site of phospholipid synthesis?

A

Smooth ER

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10
Q
  1. What is lecithin
  2. What is it important for
  3. What will happen without it
A
  1. A phospholipid
  2. Making lung surfactant in infants
  3. Atelectasis (lung collapse)
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11
Q

How do you know when a baby lung is mature?

A

When lecithin/sphingomyelin ratio is greater than 2

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12
Q

What do phospholipases do?

Name 2 phospholipases

A

Degrade glycerophospholipids

Phospholipase A2 and phospholipase C

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13
Q

Phospholipase A2

  1. Releases?
  2. What part of body has a lot of this enzyme
  3. What inhibits this enzyme?
A
  1. Arachidonic acid
  2. Pancreatic secretions
  3. Glucocorticoids
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14
Q

Phospholipase C

  1. Found where in the body?
  2. Activated by?
A
  1. Liver lysosomes

2. PIP2 (secondary messenger signaling pathway)

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15
Q

What is sphingomyelin

__ + __ = sphingomyelin

A

Phospholipid

Ceramide + phosphocholine group

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16
Q
  1. What is ceramide
  2. Where is it synthesized
  3. What is ceramide made from
  4. So precursor for ceramide is
A
  1. Precursor for glycosphingolipids
  2. Cytosolic leaflet of ER
  3. Palmitoyl-CoA + serine = sphinganine; fatty acyl CoA converts sphinganine to ceramide
  4. Sphingosine and fatty acid
17
Q

Function of sphingomyelinase

What is it necessary for

Where is this enzyme located

A

Hydrolyzes sphingomyelin (brings it back to ceramide+phosphocholine)

Important for myelin sheath

Lysosome

18
Q

Niemann-Pick disease
1. Caused from deficiency in

  1. Type A vs. Type B
  2. Disease can be detected on slide by
A
  1. Acid sphingomyelinase
  2. Type A= more rare and more severe; death by age 2-3, enlarged liver and spleen due to lipid deposit
    Type B= less severe; can be treated with bone marrow/enzyme replacements
    ~both can also give mental retardation~
  3. Zebra inclusion bodies
19
Q

Another name for glycolipids

Essential component of

Predominantly present on

A

Glycosphingolipids

All membranes (like phospholipids)

Nerve cells

20
Q

Glycolipids are antigenic what does that mean

Most important antigens for our purposes

Glycolipids also require?

A

Can have an antigen response, can cause inflammation

Blood types (A, B, AB, O) - determined by glycolipids

Ceramide

21
Q

What is the substrate for synthesis of glycolipids

A

Nucelotide sugar - usually UDP, could also be NANA (CMP-sialic acid)

22
Q

What is the difference between sphingophospholipid and sphingoglycolipid

A

Both have ceramide has hydrophobic tail but polar head will be different

23
Q

Glycolipids are degraded where ?

How?

A

Lysosome

Via endocytosis (acid glycosidases)

24
Q

What are the 4 classes of glycolipids

What makes them different

Glycolipids are important for

A

Cerebrosides, globosides, sulfatides, and gangliosides

What sugar is attached to the ceramide

Nerves/neurons/nervous system

25
Q

How to make each of the glycolipids:

  1. Cerebrosides
  2. Globosides
  3. Sulfatides
  4. Gangliosides
A
  1. Ceramide +glucose (UDP)
  2. Ceramide + 2 or more UDP
  3. Ceramide + galactose
  4. Ceramide + Globoside + CMP-NANA
26
Q

Cerebrosides are commin in

A

Myelin sheath, neurons, and maintaining integrity of skin

27
Q

Sulfatides are common in

What is unique about these

A

Nerve and kidney

These are the acidic glycolipids

28
Q

NANA is composed of

NANA is synthesized from

Reactivated by condensation with CTP to form

A

9 carbon sugar

Hexose sugar and PEP

CMP-sialic acid

29
Q

Other function of globoside/ganglioside

A

Shock absorbers of the skull

30
Q

Difference in blood type antigens

  1. O
  2. A
  3. B
A
  1. No galactose
  2. Galactose amine
  3. Galactose

~so difference depends on sugar that is attached to ceramide

31
Q

Sphingolipidoses function

Deficiency leads to

A

Breakdown of sphingolipids in lysosome

Progressive mental retardation and death in early childhood

32
Q

4 diseases of sphingolipidosis

A

Niemann pick, gauchers, tay-sachs, and fabry

33
Q

Niemann Pick

    1. Defect in
      1. Symptoms
      2. Leads to accumulation of
      3. Key symptom
A
  1. Sphingomyelinase
  2. Liver/spleen enlargement, mental retardation
  3. Sphingomyelin
  4. Cherry red spot on macula of eye
34
Q

Tay-Sachs disease - common

    1. Defect in
      1. Symptoms
      2. Leads to accumulation of
      3. Common in what kind of people
A
  1. Beta Hexosaminidase A (Hex A)
  2. Mental retardation, blindness, death by age
  3. Ganglioside Gm2
  4. Ashkenazi Jews

~also cherry red spot, but no organ involvement

35
Q

Gauchers disease - most common

    1. Defect in
      1. Symptoms
      2. Leads to accumulation of
      3. How will cell appear on histology slide
A
  1. Glucocerebrosidase
  2. Liver/spleen enlargement, osteoporosis, mental retardation in infantile form only
  3. Glucocerebroside
  4. Cell looks like crumpled tissue paper
36
Q

Fabrys disease

    1. Defect in
      1. Symptoms
      2. Leads to accumulation of
A
  1. Alpha galactosidase
  2. Skin rash, kidney failure, pain in lower extremities
  3. Trihexosylceramide

Biochemistry Block 3 (11 decks)

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