Model Tikus Omenn Syndrome
Abstract: In 1965, Gilbert
Omenn described a rare, inherited disorder which produced paradoxical
combination of immunodeficiency and immune dysregulation. The term Omenn
Syndrome was coined originating from this report. More than 40 years ago since
it first described, the pathogenesis of OS has remained mysterious and these
could be due to unavailable animal model for OS yet. This paper evaluates and
discusses 2 murine models of OS that have been found and developed recently by
2 research groups from Japan and Italia. These two murine models bearing
mutation in the V(D)J recombination analogous to those causing human OS. These
two mouse models have been recapitulate the disease and provide insight into
the pathogenesis of OS and also it relation with immunodeficiency combined with
autoimmunity and atopy in OS and other related diseases.
Penulis: Khie Khiong
Kode Jurnal: jpkedokterandd080020
