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Individuals with a fatty-acid metabolism disorder are unable to metabolize this fat source for energy, halting bodily processes. [1] Most individuals with a fatty-acid metabolism disorder are able to live a normal active life with simple adjustments to diet and medications. If left undiagnosed many complications can arise.
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.
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 .
Ketogenesis is the biochemical process through which organisms produce ketone bodies by breaking down fatty acids and ketogenic amino acids. [ 1 ] [ 2 ] The process supplies energy to certain organs, particularly the brain , heart and skeletal muscle , under specific scenarios including fasting , caloric restriction , sleep, [ 3 ] or others.
A diagrammatic illustration of the process of lipolysis (in a fat cell) induced by high epinephrine and low insulin levels in the blood. Epinephrine binds to a beta-adrenergic receptor in the cell membrane of the adipocyte, which causes cAMP to be generated inside the cell.
In the body, stores of fat are referred to as adipose tissue. In these areas, intracellular triglycerides are stored in cytoplasmic lipid droplets . When lipase enzymes are phosphorylated, they can access lipid droplets and through multiple steps of hydrolysis, breakdown triglycerides into fatty acids and glycerol.
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 .
Ketone bodies are water-soluble molecules or compounds that contain the ketone groups produced from fatty acids by the liver (ketogenesis). [1] [2] Ketone bodies are readily transported into tissues outside the liver, where they are converted into acetyl-CoA (acetyl-Coenzyme A) – which then enters the citric acid cycle (Krebs cycle) and is oxidized for energy.