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Literature summary for 3.4.21.53 extracted from
- The role of Lon-mediated proteolysis in the dynamics of mitochondrial nucleic acid-protein complexes (2017), Sci. Rep., 7, 631 .
Activating Compound
| Activating Compound | Comment | Organism | Structure |
|---|---|---|---|
| additional information | substrate binding stimulates the enzyme's ATPase activity | Saccharomyces cerevisiae | |
| additional information | substrate binding stimulates the enzyme's ATPase activity, 1.37 to 2.39fold, overview | Homo sapiens |
Cloned(Commentary)
| Cloned (Comment) | Organism |
|---|---|
| gene Lonp1 | Homo sapiens |
| gene LONP1, recombinant expression of GFP-tagged Lon in Saccharomyces cerevisiae strain BY4742 in mitochondrial nucleoids | Saccharomyces cerevisiae |
Localization
| Localization | Comment | Organism | GeneOntology No. | Textmining |
|---|---|---|---|---|
| mitochondrion | human Lon protease localizes to mitochondrial nucleoids | Homo sapiens | 5739 | - |
| mitochondrion | yeast Lon protease localizes to mitochondrial nucleoids | Saccharomyces cerevisiae | 5739 | - |
Metals/Ions
| Metals/Ions | Comment | Organism | Structure |
|---|---|---|---|
| Mg2+ | required | Homo sapiens |  |
| Mg2+ | required | Saccharomyces cerevisiae | |
Natural Substrates/ Products (Substrates)
| Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| Abf2 + H2O | Saccharomyces cerevisiae | a yeast mitochondrial protein, homologuous to human mitochondrial TFAM protein | ? | - |
? | |
| Abf2 + H2O | Saccharomyces cerevisiae ATCC 204508 | a yeast mitochondrial protein, homologuous to human mitochondrial TFAM protein | ? | - |
? | |
| Mgm101 + H2O | Saccharomyces cerevisiae | a yeast mitochondrial protein | ? | - |
? | |
| Mgm101 + H2O | Saccharomyces cerevisiae ATCC 204508 | a yeast mitochondrial protein | ? | - |
? | |
| MrpL32 + H2O | Homo sapiens | human MrpL32, a component of the 39S large subunit of the mitochondrial ribosome | ? | - |
? | |
| TFAM + H2O | Homo sapiens | only DNA-free TFAM is a substrate for human mitochondrial Lon. TFAM is released from DNA upon phosphorylation by protein kinase A, and TFAM not bound to DNA, whether phosphorylated or not, is a substrate for the ATP-dependent mitochondrial Lon protease | ? | - |
? | |
| Twinkle helicase + H2O | Homo sapiens | a human mitochondrial protein | ? | - |
? | |
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Homo sapiens | P36776 | - |
- |
| Saccharomyces cerevisiae | P36775 | - |
- |
| Saccharomyces cerevisiae ATCC 204508 | P36775 | - |
- |
Substrates and Products (Substrate)
| Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| Abf2 + H2O | a yeast mitochondrial protein, homologuous to human mitochondrial TFAM protein | Saccharomyces cerevisiae | ? | - |
? | |
| Abf2 + H2O | a yeast mitochondrial protein, homologuous to human mitochondrial TFAM protein. The substrate is protected from degradation when bound to a nucleic acid. Abf2 associates with both types of DNA, dsDNA and ssDNA | Homo sapiens | ? | - |
? | |
| Abf2 + H2O | a yeast mitochondrial protein, homologuous to human mitochondrial TFAM protein. The substrate is protected from degradation when bound to a nucleic acid. Abf2 associates with both types of DNA, dsDNA and ssDNA | Saccharomyces cerevisiae | ? | - |
? | |
| Abf2 + H2O | a yeast mitochondrial protein, homologuous to human mitochondrial TFAM protein | Saccharomyces cerevisiae ATCC 204508 | ? | - |
? | |
| Abf2 + H2O | a yeast mitochondrial protein, homologuous to human mitochondrial TFAM protein. The substrate is protected from degradation when bound to a nucleic acid. Abf2 associates with both types of DNA, dsDNA and ssDNA | Saccharomyces cerevisiae ATCC 204508 | ? | - |
? | |
| beta-casein + H2O | - |
Homo sapiens | ? | - |
? | |
| beta-casein + H2O | - |
Saccharomyces cerevisiae | ? | - |
? | |
| beta-casein + H2O | - |
Saccharomyces cerevisiae ATCC 204508 | ? | - |
? | |
| Mgm101 + H2O | a yeast mitochondrial protein | Saccharomyces cerevisiae | ? | - |
? | |
| Mgm101 + H2O | a yeast mitochondrial protein. The substrate is protected from degradation when bound to a nucleic acid | Homo sapiens | ? | - |
? | |
| Mgm101 + H2O | a yeast mitochondrial protein. The substrate is protected from degradation when bound to a nucleic acid | Saccharomyces cerevisiae | ? | - |
? | |
| Mgm101 + H2O | a yeast mitochondrial protein | Saccharomyces cerevisiae ATCC 204508 | ? | - |
? | |
| Mgm101 + H2O | a yeast mitochondrial protein. The substrate is protected from degradation when bound to a nucleic acid | Saccharomyces cerevisiae ATCC 204508 | ? | - |
? | |
| MrpL32 + H2O | human MrpL32, a component of the 39S large subunit of the mitochondrial ribosome | Homo sapiens | ? | - |
? | |
| MrpL32 + H2O | human MrpL32, a component of the 39S large subunit of the mitochondrial ribosome. The substrate is protected from degradation when bound to a nucleic acid | Homo sapiens | ? | - |
? | |
| TFAM + H2O | only DNA-free TFAM is a substrate for human mitochondrial Lon. TFAM is released from DNA upon phosphorylation by protein kinase A, and TFAM not bound to DNA, whether phosphorylated or not, is a substrate for the ATP-dependent mitochondrial Lon protease | Homo sapiens | ? | - |
? | |
| TFAM + H2O | only DNA-free TFAM is a substrate for human mitochondrial Lon. The substrate is protected from degradation when bound to a nucleic acid | Homo sapiens | ? | - |
? | |
| Twinkle helicase + H2O | a human mitochondrial protein | Homo sapiens | ? | - |
? | |
| Twinkle helicase + H2O | a human mitochondrial protein. The substrate is not protected from degradation when bound to a nucleic acid. Twinkle is degraded by hLon even in the presence of both ssDNA and dsDNA with either 3' or 5' overhangs | Homo sapiens | ? | - |
? | |
Synonyms
| Synonyms | Comment | Organism |
|---|---|---|
| hLon | - |
Homo sapiens |
| lon protease | - |
Homo sapiens |
| lon protease | - |
Saccharomyces cerevisiae |
| LONP1 | - |
Homo sapiens |
| mitochondrial Lon protease | - |
Homo sapiens |
| mitochondrial Lon protease | - |
Saccharomyces cerevisiae |
| PIM1 | - |
Saccharomyces cerevisiae |
| ScLon | - |
Saccharomyces cerevisiae |
Cofactor
| Cofactor | Comment | Organism | Structure |
|---|---|---|---|
| ATP | - |
Homo sapiens | |
| ATP | - |
Saccharomyces cerevisiae | |
General Information
| General Information | Comment | Organism |
|---|---|---|
| physiological function | the mitochondrial Lon protease has been identified as an integral nucleoid core factor in human mitochondria, it is involved in selective protein turnover (including ribosomal proteins)12 and the ATP-dependent degradation of misfolded or damaged mitochondrial proteins. It also has a chaperone-like function in the assembly of certain mitochondrial complexes, which persists even if its proteolytic activity is impaired. Role of Lon-mediated proteolysis in the dynamics of mitochondrial nucleic acid-protein complexes. The mitochondrial Lon protease could be involved in the regulation of such fundamental processes as nucleoid packaging, mtDNA replication, mtDNA maintenance and recombination, and the assembly of mitochondrial ribosomes | Homo sapiens |
| physiological function | the mitochondrial Lon protease has been identified as an integral nucleoid core factor in human mitochondria, it is involved in selective protein turnover (including ribosomal proteins)12 and the ATP-dependent degradation of misfolded or damaged mitochondrial proteins. It also has a chaperone-like function in the assembly of certain mitochondrial complexes, which persists even if its proteolytic activity is impaired. Role of Lon-mediated proteolysis in the dynamics of mitochondrial nucleic acid-protein complexes. The mitochondrial Lon protease could be involved in the regulation of such fundamental processes as nucleoid packaging, mtDNA replication, mtDNA maintenance and recombination, and the assembly of mitochondrial ribosomes | Saccharomyces cerevisiae |
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