Peroxisomal disorder

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Definition

noun, plural: peroxisomal disorders

A type of metabolic disorder caused by a dysfunctional peroxisome


Supplement

Metabolic disorders refer to the various disorders in the normal metabolism and are usually associated with pathological mutation in the gene that codes for a particular protein or enzyme. The mutation in a gene may cause the alteration of the structure and function of the protein it codes for. Thus, this mutation may impair a particular metabolic process. There are many types of metabolic disorders and one of them is peroxisomal disorder.

Peroxisomal disorder is a metabolic disorder caused by dysfunctional peroxisome. Peroxisome is an organelle that catabolizes very long chain fatty acids, polyamines, hydrogen peroxide, etc. It is also involved in the synthesis of plasmalogens. A defective peroxisome leads to various disorders and medically important conditions associated with defective peroxisome are collectively called peroxisomal disorders. Examples of these disorders are as follows:

The underlying cause of peroxisomal disorders are due to mutations in genes affecting the peroxisome functions, such as enzymes involved in peroxisome metabolic pathways and peroxins (i.e. proteins involved in peroxisome assembly and biogenesis) encoded by PEX genes.


Synonyms:

  • peroxisomal disease
  • peroxisome disease

See also: