Lipid Storage Diseases

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.