What is alpha oxidation?

Alfa or A-oxidation is a name assigned to the enzymatic shortening of fatty acid derivatives containing the methyl group (ch 3 -) connected to the carbon atom of the located beta (β) to the carboxylic group (-COO -). Alpha oxidation involves the metabolization of specific fatty acids only one of a number of processes in the peroxisomes of eukaryotic cells. Insufficient alpha oxidation in the human body leads to a serious accumulation of phytanic acid, which is closely associated with refsum disease. It is known that this genetic disorder produces a number of disorders, many of which do not threaten life. Most cases of Refsum are associated with mutations in the Phyh gene. Out and ch _{3 attached to β -carbonatoma is written as "I" - standing for methyl - for simplifying visualization. Identification of carbon atoms (C) -Z Rights to the left, not counting coenzyme carbons CO carbon atoms to the left of the Y position are excluded for simplification. The biological process requires an enzyme because the methyl connectionSpecifically, it prevents metabolism at Beta.}

In the first step in a multi-stage process, the hydrogen atom is replaced by a hydroxyl group (-OH) in the A-U-ulik. The result is the · · ch ₂

ch (me) CH (OH) -coo- (CoA). This is followed by decarboxylation associated with oxidation, to form · ' ch 2 ch (me) -coo- (coa). After alpha oxidation, another metabolic steppe called beta oxidation. Unfortunately, failure in the alpha oxidation process is not covered by this process - phytanic acid is not metabolized by beta oxidation or other metabolic metabolic metabolistics of fatty acids.

Since the disease refsum is closely related to the excess of phytanic acid and phytanic acid comes directly from the diet, a large part of the individual treatment is related to the diet. It is specifically found in dairy products, beef, lamb and some fish - including tunaKa, cod and cod. In some individuals, a difficult and expensive blood filtration procedure called plasmapheresis can be used. Smaller itchy problems with skin degeneration and muscle degeneration can be solved by treatment and one day the enzymatic treatment can reduce the accumulation of phytanic acid and what is refsum. At present, however, the result may be as serious as the death of cardiac arrrhythmia.