Zebrafish: A Model System to Study Heritable Skin Diseases

Abstract

Heritable skin diseases represent a broad spectrum of clinical manifestations due to mutations in ~500 different genes. A number of model systems have been developed to advance our understanding of the pathomechanisms of genodermatoses. Zebrafish (Danio rerio), a freshwater vertebrate, has a well-characterized genome, the expression of which can be easily manipulated. The larvae develop rapidly, with all major organs having developed by 5–6 days post fertilization, including the skin, consisting of the epidermis comprising two cell layers and separated from the dermal collagenous matrix by a basement membrane. This perspective highlights the morphologic and ultrastructural features of zebrafish skin, in the context of cutaneous gene expression. These observations suggest that zebrafish provides a useful model system to study molecular aspects of skin development, as well as the pathogenesis and treatment of select heritable skin diseases.

Premature Termination Codon Read-Through in the ABCC6 Gene: Potential Treatment for Pseudoxanthoma Elasticum

“…In order to test the functionality of the full-length ABCC6 protein synthesized in the presence of PTC124, which instead of an arginine in position 1141 could contain cysteine, tryptophan or glycine, we utilized a zebrafish mRNA rescue system recently developed by us (Li et al , 2011). In this system, injection of 1 to 4-cell zebrafish embryos with a morpholino corresponding to the exon 7/intron 7 border of the abcc6a gene decreases the gene expression by up to 80% and induces a phenotype of pericardial edema and curled tail associated with early demise.…”

Curcumin-encapsulated nanoparticles as innovative antimicrobial and wound healing agent

“…Furthermore, the data presented recapitulate the utility of the embryonic zebrafish model to study skin pathology and comment on its physiologic relevance to humans as suggested by previous investigators. 30 …”

Molecular Therapeutics for Heritable Skin Diseases

“…In addition to transgenic animals, human xenograft transplants onto immunocompromised mice have provided useful model systems (Siprashvili et al , 2010). Finally, to our knowledge, previously unreported vertebrate model systems, such as zebrafish, have been explored as potential models for heritable skin diseases (Li et al , 2011). …”