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Literature summary for 3.4.24.B18 extracted from

  • Nolden, M.; Ehses, S.; Koppen, M.; Bernacchia, A.; Rugarli, E.I.; Langer, T.
    The m-AAA protease defective in hereditary spastic paraplegia controls ribosome assembly in mitochondria (2005), Cell, 123, 277-289.
    View publication on PubMed
Search for more literature for 3.4.24.B18

Protein Variants

Protein Variants Comment Organism
E559Q of subunit Yta10, enzyme is still capable of substrate binding but lacks proteolytic activity Saccharomyces cerevisiae
E614Q of subunit Yta12, enzyme is still capable of substrate binding but lacks proteolytic activity Saccharomyces cerevisiae
additional information deletion of either subunit Yta10 or Yta12, synthesis of mitochondrially encoded proteins is strongly impaired Saccharomyces cerevisiae
additional information deletion of subunit paraplegin, maturation of yeast MrpL32 protein is impaired. Functional conservation of yeast Yta10/Yta12 and murine paraplegin/Afg3L2 Mus musculus

Localization

Localization Comment Organism GeneOntology No. Textmining
mitochondrion
-
 Mus musculus 5739
-
mitochondrion
-
 Saccharomyces cerevisiae 5739
-

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
MrpL32 + H2O Saccharomyces cerevisiae mitochondrial ribosomal protein, enzyme mediates processing of newly imported MrpL32. Maturation in vivo depends on enzyme ?
-
 ?

Organism

Organism UniProt Comment Textmining
Mus musculus
-
 subunits paraplegin and Afg3L2
-
Saccharomyces cerevisiae
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
liver
-
 Mus musculus
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
MrpL32 + H2O mitochondrial ribosomal protein, enzyme mediates processing of newly imported MrpL32. Maturation in vivo depends on enzyme Saccharomyces cerevisiae ?
-
 ?
MrpL32 + H2O mitochondrial ribosomal protein, enzyme mediates processing of newly imported MrpL32 Saccharomyces cerevisiae ?
-
 ?
MrpL32 + H2O yeast mitochondrial ribosomal protein Mus musculus ?
-
 ?

Subunits

Subunits Comment Organism
More interaction of enzyme with prohibitin proteins Phb1 and Phb2 of the inner mitochondrial membrane Saccharomyces cerevisiae