University:
Liberty University
Course:
BIOM 500 | Introduction to Biomedical Sciences
Academic year:

2024
Views:

233

Pages:

6
Author:

Sheha A.

Refsum's Disease It is caused due to the absence of an enzyme phytanoyl-CoA hydroxylase. This leads to the accumulation of phytanic acid in blood. Symptoms: Neurological symptoms such as ataxia and retinitis pigmentosa. Unexplained deafness. Cause: There is a deficiency of phytanoyl-CoA hydroxylase enzyme. This enzyme is needed to convert phytanic acid to pristanic acid. Both phytanic acid and pristanic acid are oxidized to form propionyl CoA. The propionyl CoA then enters the TCA cycle. Peroxisomal Fatty Acid Oxidation This system oxidizes very long chain fatty acids (VLCFAs). This is a modified form of β-oxidation that can lead to the formation of acetyl-CoA and hydrogen peroxide (H2O2). This oxidation is not linked to ATP production. Peroxisomal β-oxidation This system does not attack double bonds in unsaturated fatty acids. This system can also oxidize branched chain fatty acids. This system takes place in the peroxisomes, cholesterol biosynthesis, and bile acid formation. Lipogenesis When excess glucose is converted into fats, it is generally converted into fatty acids. These are then stored in the form of triglycerides. De Novo Synthesis of Fatty Acids It is also called as fatty acid synthesis. This process is mainly occurs in cytosol. Palmitic Acid Palmitic acid is the major fatty acid synthesized. These fatty acids are major components of triglycerides. These are stored in liver, adipose tissue, etc. Statins: Cholesterol synthesis inhibitors. End product: Palmitic acid.