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2x36 is a 6 chain structure with sequence from Human. This domain belongs to the [1] Lon protease family.

Mitochondrial Lon protease is an ATP-dependent serine protease involved in the selective degradation of abnormal proteins. LONP1 situated on chromosome 19 is the nuclear gene encoding mitochondrial Lon protein. The single species of mRNA of this protein is found in the mitochondrial matrix. This protein from human tissues has a molecular mass of 100 kDA.

Function

The mitochondrial Lon protease is an important regulator of mitochondrial metabolism including the maintenance and repair of mitochondrial DNA. This protein is also essential for homeostasis of mitochondria, and by regulating some regulatory proteins which have a short life or damaged proteins.

Lon protease has three main roles.

This protein is able to do a proteolytic digestion of oxidized proteins which allows the renewal of essential mitochondrial enzymes such as aconitase or Mitochondrial transcription factor A. Lon protease is involved in mtDNA replication and mitogenesis by being a mitochondrial DNA-bing protein. Mitochondrial Lon protease interacts with protein chaperone, notably HSP60-Hsp70 complex to protect cell from apoptosis under environmental stress^[3].

The mitochondrial Lon protease is essentially found in the cytoplasmic of mitochondria because amino-acid has a potential mitochondrial targetting presequences.

General structure

Structural highlights

Evolutionary conservation

Disease

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