Lon peptidase 2, peroxisomal

Lon peptidase 2, peroxisomal is a protein that in humans is encoded by the LONP2 gene. This protein is a member of the Lon protease family, which is characterized by the presence of an ATP-dependent serine protease domain.

Function

Lon peptidase 2, peroxisomal is a protease that is involved in the selective degradation of misfolded, damaged or short-lived proteins in the peroxisome, a type of organelle found in virtually all eukaryotic cells. The protein is also involved in the maturation of specific proteins in the peroxisome after they have been imported.

Clinical significance

Mutations in the LONP2 gene have been associated with a number of diseases, including Peroxisome Biogenesis Disorder, a group of genetic disorders that affect the normal functioning of peroxisomes.

Structure

The structure of Lon peptidase 2, peroxisomal includes a serine protease domain, an ATPase domain, and a substrate-binding domain. The protein is located in the matrix of the peroxisome.

See also

• Lon protease family
• Peroxisome
• Protease
• Gene

References

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