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The enzyme or transport protein can be missing or improperly constructed, resulting in it not working. This leaves the body unable to produce energy within the liver and muscles from fatty acid sources. [1] The body's primary source of energy is glucose; however, when all the glucose in the body has been expended, a normal body digests fats.
Adipose tissue (also known as body fat or simply fat) is a loose connective tissue composed mostly of adipocytes. [ 1 ] [ 2 ] It also contains the stromal vascular fraction ( SVF ) of cells including preadipocytes , fibroblasts , vascular endothelial cells and a variety of immune cells such as adipose tissue macrophages .
Over time, the buildup of fats may cause permanent cellular and tissue damage, particularly in the brain, peripheral nervous system, liver, spleen, and bone marrow. Inside cells under normal conditions, lysosomes convert, or metabolize, lipids and proteins into smaller components to provide energy for the body.
A large meta-analysis has shown that white adipose tissue cell size is dependent on measurement methods, adipose tissue depots, age, and body mass index; for the same degree of obesity, increases in fat cell size were also associated with the dysregulations in glucose and lipid metabolism. [2]
Lipodystrophy syndromes are a group of genetic or acquired disorders in which the body is unable to produce and maintain healthy fat tissue. [ 1 ] [ 2 ] The medical condition is characterized by abnormal or degenerative conditions of the body's adipose tissue .
When the body has excess carbohydrates available, some glucose is fully metabolized, and some of it is stored in the form of glycogen or, upon citrate excess, as fatty acids (see lipogenesis). Coenzyme A is recycled at this step. When the body has no free carbohydrates available, fat must be broken down into acetyl-CoA in order to get energy.
Fatty acids are stored as triglycerides in the fat depots of adipose tissue. Between meals they are released as follows: Between meals they are released as follows: Lipolysis , the removal of the fatty acid chains from the glycerol to which they are bound in their storage form as triglycerides (or fats), is carried out by lipases .
Additional symptoms that occur throughout the body can be attributed to the lack of sufficient lipid sources. [4] Chronic Malabsorptive Diarrhea- Diarrhea that results from the poor absorption of fats; Steatorrhea- Abnormal stools, often foul smelling, due to the increased presence of undigested fats; Vomiting