Lipid Storage Diseases

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.

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.