Lipid Storage Diseases and Disorders
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Questions and Answers

What is the primary cause of Respiratory Distress Syndrome in preterm newborns?

  • Deficiency of sphingomyelinase
  • Absence of sphingomyelin
  • Deficiency of dipalmitoyl lecithin (correct)
  • Excess of sphingolipids
  • Which enzyme defect is commonly associated with Sphingolipidoses?

  • Thermogenin
  • Hemoglobinase
  • Glycerol kinase
  • Hexosaminidase A (correct)
  • What differentiates brown adipose tissue from white adipose tissue in terms of lipid droplets?

  • Brown adipose tissue contains smaller lipid droplets than white adipose tissue (correct)
  • Both contain the same size of lipid droplets
  • Brown adipose tissue contains larger lipid droplets than white adipose tissue
  • White adipose tissue contains smaller lipid droplets than brown adipose tissue
  • Which protein in brown adipose tissue uncouples oxidation from phosphorylation?

    <p>Thermogenin (UCP1)</p> Signup and view all the answers

    Where is brown adipose tissue mainly located in humans?

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

    Which of the following statements is true about Multiple Sclerosis?

    <p>It is a demyelinating disease characterized by loss of phospholipids from white matter</p> Signup and view all the answers

    Why does brown adipose tissue appear brown?

    <p>Due to the higher number of mitochondria with more iron content</p> Signup and view all the answers

    Which of the following statements is most accurate regarding brown adipose tissue's role in energy metabolism?

    <p>Brown adipose tissue produces more heat and less ATP</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 diseases and disorders related to lipid storage, including Respiratory Distress Syndrome, Multiple Sclerosis, and Sphingolipidoses.

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