• Publications
  • Influence
Functions of KLK4 and MMP-20 in dental enamel formation
The principle functions of MMP-20 and KLK4 in dental enamel formation are to facilitate the orderly replacement of organic matrix with mineral, generating an enamel layer that is harder, less porous, and unstained by retained enamel proteins.
Dentin Sialophosphoprotein Is Processed by MMP-2 and MMP-20 in Vitro and in Vivo*
Exact correspondence between DSPP cleavage sites that occur in vivo and those generated in vitro demonstrates that M MP-2 and MMP-20 process D SPP into smaller subunits in the dentin matrix during odontogenesis.
Enamel Defects and Ameloblast-specific Expression in Enam Knock-out/lacZ Knock-in Mice*
Ameloblast-specific expression of enamelin is demonstrated and it is revealed that enamin is essential for proper enamel matrix organization and mineralization.
Hypomaturation Enamel Defects in Klk4 Knockout/LacZ Knockin Mice*
Results demonstrate that Klk4 is essential for the removal of enamel proteins and the proper maturation ofEnamel crystals and an important finding was that individual enamel crystallites of erupted teeth failed to grow together, interlock, and function as a unit.
Expression, Structure, and Function of Enamel Proteinases
  • J. Simmer, J. Hu
  • Chemistry, Medicine
    Connective tissue research
  • 1 January 2002
Two major enamel proteinases have been identified: enamelysin (MMP20) and kallikrein 4 (KLK4).
FAM20A Mutations Can Cause Enamel-Renal Syndrome (ERS)
FAM20A, which has a kinase homology domain and localizes to the Golgi, is a putative Golgi kinase that plays a significant role in the regulation of biomineralization processes, and that mutations in FAM20A cause both AIGFS and ERS.
A nonsense mutation in the enamelin gene causes local hypoplastic autosomal dominant amelogenesis imperfecta (AIH2).
The results show that while a splice site mutation is associated with smooth and thin hypoplastic AI, a base substitution resulting in a shorter peptide causes local hypoplasia of the enamel, a milder form of AI.
FAM83H mutations in families with autosomal-dominant hypocalcified amelogenesis imperfecta.
This work has studied two families with autosomal-dominant hypocalcified AI and identified nonsense mutations (R325X and Q398X) in the FAM83H gene on chromosome 8q24.3, demonstrating that FAM 83H is required for proper dental-enamel calcification.
How do enamelysin and kallikrein 4 process the 32-kDa enamelin?
This work identified the cleavage sites of 32-kDa enamelin that are generated by proteolysis with MMP-20 and KLK4 and characterized them by Edman sequencing, amino acid analysis, and mass spectrometry.
Mutational hot spot in the DSPP gene causing dentinogenesis imperfecta type II
Findings suggest that a single mutation at the first nucleotide of exon 3 of the DSPP gene represents a mutational “hot spot” in the D SPP gene and supports the conclusion that D GI type II and DGI type III are not separate diseases but rather the phenotypic variation of a single disease.