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Literature summary for 3.4.21.109 extracted from

  • Sales, K.U.; Masedunskas, A.; Bey, A.L.; Rasmussen, A.L.; Weigert, R.; List, K.; Szabo, R.; Overbeek, P.A.; Bugge, T.H.
    Matriptase initiates activation of epidermal pro-kallikrein and disease onset in a mouse model of Netherton syndrome (2010), Nat. Genet., 42, 676-683.
    View publication on PubMedView publication on EuropePMC

Localization

Localization Comment Organism GeneOntology No. Textmining
membrane
-
Mus musculus 16020
-

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
prostasin + H2O Mus musculus
-
?
-
?

Organism

Organism UniProt Comment Textmining
Mus musculus
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
epidermis matriptase is detected in the suprabasal epidermis with the highest level of expression in the granular and transitional cell layers Mus musculus
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
prostasin + H2O
-
Mus musculus ?
-
?

General Information

General Information Comment Organism
malfunction the genetic elimination of matriptase completely abolishes the aberrant caseinolytic activity that is caused by lympho-epithelial Kazal-type-related inhibitor (LEKTI) deficiency in both the lower epidermal layers as well as in the dermis. Matriptase elimination prevents spontaneous stratum corneum loss, restores keratohyalin granules, and improves the barrier function of LEKTI-deficient epidermis. Loss of matriptase restores corneodesmosome integrity to LEKTI-deficient epidermis. Matriptase ablation prevents kallikrein hyperactivity-mediated inflammation caused by LEKTI-deficiency Mus musculus
physiological function matriptase initiates Netherton syndrome in a lympho-epithelial Kazal-type-related inhibitor (LEKTI)-deficient mouse model by premature activation of a pro-kallikrein-related cascade. Matriptase is an efficient activator of epidermal pro-kallikreins that co-localize with LEKTI at the granular-transitional layer boundary Mus musculus