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Literature summary for 3.4.24.80 extracted from

  • Sakr, M.; Li, X.-Y.; Sabeh, F.; Feinberg, T.Y.; Tesmer, J.J.G.; Tang, Y.; Weiss, S.J.
    Tracking the Cartoon mouse phenotype hemopexin domain-dependent regulation of MT1-MMP pericellular collagenolytic activity (2018), J. Biol. Chem., 293, 8113-8127 .
    View publication on PubMedView publication on EuropePMC

Cloned(Commentary)

Cloned (Comment) Organism
gene MMP14, recombinant expression of wild-type and mutant enzymes in COS-1 cells Mus musculus

Protein Variants

Protein Variants Comment Organism
S466P naturally occuring mutation causing the Cartoon phenotype, Cartoon mice harbor the single point mutation in the MT1-MMP hemopexin domain, a 200-amino acid segment that is thought to play a critical role in regulating MT1-MMP collagenolytic activity Mus musculus

Localization

Localization Comment Organism GeneOntology No. Textmining
cell surface
-
Mus musculus 9986
-
membrane
-
Mus musculus 16020
-
additional information the enzyme traffics from the endoplasmic reticulum to the trans-Golgi network (ER 3 trans-Golgi network), where it undergoes processing to its mature form, mobilizes to the cell surface, and expresses type I collagenolytic activity Mus musculus
-
-

Metals/Ions

Metals/Ions Comment Organism Structure
Zn2+ zinc-dependent endopeptidase Mus musculus

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
proMMP-2 + H2O Mus musculus
-
MMP-2 + MMP-2 pro-peptide
-
?

Organism

Organism UniProt Comment Textmining
Mus musculus P53690
-
-

Posttranslational Modification

Posttranslational Modification Comment Organism
proteolytic modification the proenzyme undergoes processing to its mature form in the endoplasmic reticulum Mus musculus

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
proMMP-2 + H2O
-
Mus musculus MMP-2 + MMP-2 pro-peptide
-
?

Synonyms

Synonyms Comment Organism
MT1-MMP
-
Mus musculus

General Information

General Information Comment Organism
malfunction Cartoon mice harbor the single point mutation S466P in the MT1-MMP hemopexin domain, a 200-amino acid segment. The S466P substitution generates a misfolded, temperature-sensitive mutant that is abnormally retained in the endoplasmic reticulum (ER). The MT1-MMPS466P mutation replicates the phenotypic status of Mt1-mmp-null animals as well as the functional characteristics of MT1-MMP-/- cells. The wild-type hemopexin domain does not play a required role in regulating MT1-MMP trafficking, as a hemopexin domain-deletion mutant is successfully mobilized to the cell surface and displays nearly normal collagenolytic activity. Cartoon mice exhibit a pattern of stunted growth, kyphosis, and rounded skulls, Cartoon mouse phenotype, detailed overview. Cartoon mouse fibroblasts are devoid of pericellular collagenolytic activity Mus musculus