Lipid Storage Diseases
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Questions and Answers

Which deficiency mainly causes Respiratory Distress Syndrome in preterm newborns?

  • Myelin
  • Glycerol kinase
  • Dipalmitoyl lecithin (correct)
  • Sphingolipids
  • In Multiple Sclerosis, which type of lipids are primarily lost from the white matter of the nerve?

  • Triglycerides
  • Fatty acids
  • Sphingolipids
  • Phospholipids (correct)
  • What characterizes Sphingolipidoses?

  • Excessive phospholipids in the tissues
  • Loss of dipalmitoyl lecithin
  • Reduced number of mitochondria
  • Abnormal quantities of sphingolipids in the tissues (correct)
  • Which tissue type contains Thermogenin (UCP1)?

    <p>Brown adipose tissue</p> Signup and view all the answers

    In the mitochondria of brown adipose tissue, what is the relationship between oxidation and phosphorylation?

    <p>They are not coupled</p> Signup and view all the answers

    Where is brown adipose tissue primarily located in humans?

    <p>In the upper back and mediastinum</p> Signup and view all the answers

    What is a distinguishing feature of brown adipose tissue compared to white adipose tissue?

    <p>Smaller lipid droplets</p> Signup and view all the answers

    What recent finding is related to the activity of brown adipose tissue?

    <p>Inverse relation to body fat content</p> Signup and view all the answers

    Study Notes

    Lipid Storage Diseases

    • Respiratory Distress Syndrome is a condition that occurs mainly in preterm newborns, caused by a deficiency of dipalmitoyl lecithin, a major component of lung surfactant.
    • Administration of surfactant is of therapeutic benefit in Respiratory Distress Syndrome.
    • Multiple Sclerosis is a demyelinating disease characterized by a loss of lipids, mainly phospholipids, from the white matter of the nerve.
    • Sphingolipidoses are inherited diseases characterized by abnormal quantities of lipids, mainly sphingolipids, in the tissues, often in the nervous system, due to an enzyme defect.

    Brown Adipose Tissue (Brown Fat)

    • Brown adipose tissue is a type of adipose tissue that is present in hibernating mammals, newborn humans, and to a lesser extent in fit adults, where it is responsible for "diet-induced thermogenesis".
    • Brown adipose tissue is reduced or absent in obese persons.
    • Brown adipose tissue differs from white adipose tissue in several ways:
      • It contains smaller lipid droplets.
      • It contains a higher number of mitochondria with more iron content, making it brown.
      • It contains more capillaries.
      • It contains thermogenin (uncoupling protein, UCP1), which allows for oxidation and phosphorylation to be uncoupled, producing heat and little ATP.
      • It has higher activity of glycerol kinase enzyme.
    • Brown adipose tissue is located mainly in the upper back and in the mediastinum, whereas white adipose tissue is located mainly around the internal organs.
    • The activity of brown adipose tissue is inversely related to body fat content, making it a potential target for the treatment of obesity.

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    Description

    This quiz covers various lipid storage diseases, including Respiratory Distress Syndrome, Multiple Sclerosis, and Sphingolipidoses. Learn about the causes, characteristics, and treatments of these lipid-related disorders.

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