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Literature summary for 3.4.21.109 extracted from

  • Desilets, A.; Beliveau, F.; Vandal, G.; McDuff, F.O.; Lavigne, P.; Leduc, R.
    Mutation G827R in matriptase causing autosomal recessive Ichthyosis with hypotrichosis yields an inactive protease (2008), J. Biol. Chem., 283, 10535-10542.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 3.4.21.109

Cloned(Commentary)

Cloned (Comment) Organism
expression of mutant S805A in BT549 breast cancer cells, expression of truncated wild-type zymogen and mutant G287R, comprising residues 596-855 and consisting of the C-terminal end of the fourth LDLRA domain, the activation domain, and the catalytic domain, in Escherichia coli in inclusion bodies, expression of wild-type enzyme in HEK-293 cells Homo sapiens

Protein Variants

Protein Variants Comment Organism
G827R the mutation in the catalytic domain causes autosomal recessive Ichthyosis with hypotrichosis, the G827R mutant is catalytically inactive and does not perform autoproteolysis due to a blockade of access to the binding/catalytic cleft of the enzyme, molecular modeling, overview, elevated expression levels compared to the wild-type enzyme, the G827R substitution does not impair the ability of the protease to localize at the cell surface Homo sapiens
additional information generation of the 26 kDa soluble form in matriptase expressing cells is not strictly autocatalytic and may involve other proteases Homo sapiens
S805A mutation of a catalytic residue, inactive mutant, elevated expression levels compared to the wild-type enzyme Homo sapiens

Inhibitors

Inhibitors Comment Organism Structure
hepatocyte growth factor activator inhibitor-1 HAI-1, without HAI-1 active matriptase may become unstable, leading to its degradation and low protein expression Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
cell surface
-
 Homo sapiens 9986
-
extracellular
-
 Homo sapiens
-
-
intracellular
-
 Homo sapiens 5622
-
membrane a type II transmembrane serine protease Homo sapiens 16020
-

Molecular Weight [Da]

Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
80000
-
 x * 95000, full-length transmembrane isoform, SDS-PAGE, x * 80000, Gly149-early processed isoform, SDS-PAGE, x * 26000-29000, activated recombinant truncated wild-type catalytic domain, SDS-PAGE Homo sapiens
95000
-
 x * 95000, full-length transmembrane isoform, SDS-PAGE, x * 80000, Gly149-early processed isoform, SDS-PAGE, x * 26000-29000, activated recombinant truncated wild-type catalytic domain, SDS-PAGE Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
additional information Homo sapiens the G827R mutation in patients with autosomal recessive ichthyosis with hypotrichosis leads to the expression of an inactive protease ?
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Posttranslational Modification

Posttranslational Modification Comment Organism
proteolytic modification autocatalytic cleavage from the zymogen to the active form, autocatalytic activation cleavage at Arg614, and processing at the Gly149 site after which the enzyme remains associated with the membrane, the recombinant refolded truncated wild-type zymogen is capable to autoactivate, overview Homo sapiens

Purification (Commentary)

Purification (Comment) Organism
recombinant truncated wild-type zymogen and mutant G287R from Escherichia coli Homo sapiens

Reaction

Reaction Comment Organism Reaction ID
cleaves various synthetic substrates with Arg or Lys at the P1 position and prefers small side-chain amino acids, such as Ala and Gly, at the P2 position Asp771, His656, and Ser805 are the residues of the catalytic triad Homo sapiens
a

Renatured (Commentary)

Renatured (Comment) Organism
recombinant truncated wild-type zymogen and mutant G287R from Escherichia coli inclusion bodies Homo sapiens

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
benzyloxycarbonyl-Gln-Ala-Arg-7-amido-4-methylcoumarin + H2O
-
 Homo sapiens benzyloxycarbonyl-Gln-Ala-Arg + 7-amino-4-methylcoumarin
-
 ?
additional information the G827R mutation in patients with autosomal recessive ichthyosis with hypotrichosis leads to the expression of an inactive protease Homo sapiens ?
-
 ?
additional information the enzyme performs autoproteolysis Homo sapiens ?
-
 ?

Subunits

Subunits Comment Organism
? x * 95000, full-length transmembrane isoform, SDS-PAGE, x * 80000, Gly149-early processed isoform, SDS-PAGE, x * 26000-29000, activated recombinant truncated wild-type catalytic domain, SDS-PAGE Homo sapiens
More generation of the 26 kDa soluble form in matriptase expressing cells is not strictly autocatalytic and may involve other proteases, molecular modeling of wild-type and mutant G287R active site structures Homo sapiens

Synonyms

Synonyms Comment Organism
More the enzyme is a member of the type II transmembrane serine protease family Homo sapiens

Temperature Optimum [°C]

Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
37
-
 assay at Homo sapiens

pH Optimum

pH Optimum Minimum pH Optimum Maximum Comment Organism
9
-
 assay at Homo sapiens