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Literature summary for 3.4.22.34 extracted from

  • Chan, C.B.; Abe, M.; Hashimoto, N.; Hao, C.; Williams, I.R.; Liu, X.; Nakao, S.; Yamamoto, A.; Li, S.Y.; Hara-Nishimura, I.; Asano, M.; Ye, K.
    Mice lacking asparaginyl endopeptidase develop disorders resembling hemophagocytic syndrome (2009), Proc. Natl. Acad. Sci. USA, 106, 468-473.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 3.4.22.34

Activating Compound

Activating Compound Comment Organism Structure
additional information asparaginyl endopeptidase activation is autocatalytic and requires sequential removal of C- and N-terminal propeptides at different pH thresholds Mus musculus

Cloned(Commentary)

Cloned (Comment) Organism
AEP -/-, +/- and +/+ mice on a mixed 129/Ola and C57BL/6 background are generated Mus musculus

Localization

Localization Comment Organism GeneOntology No. Textmining
endosome
-
 Mus musculus 5768
-
lysosome
-
 Mus musculus 5764
-

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
additional information Mus musculus asparaginyl endopeptidase plays a pivotal role in the endosome and lysosomal degradation system and is implicated in antigen processing and presentation ?
-
 ?
additional information Mus musculus asparaginyl endopeptidaseprotein substrates on the C-terminal side of asparagine ?
-
 ?

Organism

Organism UniProt Comment Textmining
Mus musculus
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
kidney
-
 Mus musculus
-

Specific Activity [micromol/min/mg]

Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
additional information
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 asparaginyl endopeptidase deficiency provokes extramedullary hematopoiesis in the spleen and abnormally enlarged histiocytes with ingested red blood cells in bone marrow Mus musculus
additional information
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 asparaginyl endopeptidase depletion evidently enhances the phagocytic activity, suggesting that phagocytic activity of macrophages in asparaginyl endopeptidase-/- mice is elevated Mus musculus
additional information
-
 disruption of asparaginyl endopeptidase leads to late endosomes and lysosomes augmentation and dislocation from the apical region of the kidney-proximal tubule cells and the abnormal lysosomes contained in electron-dense or membranous materials Mus musculus
additional information
-
 in contrast to the enhanced macrophage activity, the natural killer cell activity in the asparaginyl endopeptidase-null animal is significantly reduced Mus musculus
additional information
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 mutant mice lacking asparaginyl endopeptidase develop fever, cytopenia, hepatosplenomegaly and hemophagocytosis, which are primary pathological manifestations of hemophagocytic syndrome/hemophagocytic lymphohistiocytosis Mus musculus
additional information
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 myeloid cells are also increased in the asparaginyl endopeptidase-null mice, e.g. 10.2% in asparaginyl endopeptidase-null mice versus 1.2% in wild-type Mus musculus
additional information
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 red blood cells from asparaginyl endopeptidase-null mice are defective in plasma membrane components Mus musculus
additional information
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 the liver is significantly enlarged in asparaginyl endopeptidase deficient mice as compared with the normal counterparts Mus musculus
additional information
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 the percentage of Ter-119+/CD45+ erythroid lineage cells in the spleen is increased in the asparaginyl endopeptidase-deficient mice, e.g. 18.2% in a representative asparaginyl endopeptidase-null mouse compared with just 0.4% in a wild type control Mus musculus
additional information
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 the processing of the lysosomal proteases cathepsins in kidney is completely defective in asparaginyl endopeptidase-deficient mice with accumulation of macromolecules in the lysosomes, which is typically seen in lysosomal disorders Mus musculus
additional information
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 the spleens from asparaginyl endopeptidase-null mice are 5-10times larger than those of wild type controls Mus musculus

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
additional information asparaginyl endopeptidase plays a pivotal role in the endosome and lysosomal degradation system and is implicated in antigen processing and presentation Mus musculus ?
-
 ?
additional information asparaginyl endopeptidaseprotein substrates on the C-terminal side of asparagine Mus musculus ?
-
 ?

Synonyms

Synonyms Comment Organism
Asparaginyl endopeptidase
-
 Mus musculus
legumain
-
 Mus musculus

pH Range

pH Minimum pH Maximum Comment Organism
additional information
-
 asparaginyl endopeptidase activation requires sequential removal of C- and N-terminal propeptides at different pH thresholds Mus musculus