Core - Animal

Klement, John F.

Abstract

The Dermatology Animal Core (DAC). DAC was established in 1996 to generate transgenic mice of dermatological interest. Resources include a full microinjection suite for both pronuclear and blastocyst injections and the mice necessary to generate transgenic animals using, these protocols. Typically, we use FVB/N or CD- 1 mice for pronuclear injection. C57BL/6J mice are used as donor blastocysts for ES microinjection. Either FVB/N or CD- 1 vasectornized males are used to mate with B6CBAF1 (F1 generation of a cross between B6 and CBA mice) females to generate the pseudopregnant foster mothers. Currently, DAC consists of I faculty member (John F. Klement; 30% effort) as the director and 1 technician. By itself, or with DNA targeting vectors generated in other labs, DAC has successfully generated three knock-out lines. These include the collagen type VII null mice, which serves as a model for recessive dystrophic epidermolysis bullosa, and also periplakin, and ladinin. In collaboration with members of the Department of Dermatology, who generated the recombinant ES cells, DAC generated three other gene ablation mouse lines. Currently, DAC has several projects in progress. DAC has successfully generated several mouse lines by pronuclear injection. These mice are currently being analyzed.

Funding Agency

• Agency: National Institute of Health (NIH)
• Institute: National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
• Type: Research Program Projects (P01)
• Project #: 2P01AR038923-16
• Application #: 6580310
• Project Start: 2002-04-01
• Project End: 2007-03-31
• Support Year: 16
• Fiscal Year: 2002

Institution

• Name: Thomas Jefferson University
• DUNS #: 061197161
• City: Philadelphia
• State: PA
• Country: United States
• Zip Code: 19107

Publications

Chung, Hye Jin; Uitto, Jouni (2010) Epidermolysis bullosa with pyloric atresia. Dermatol Clin 28:43-54

Chung, Hye Jin; Uitto, Jouni (2010) Type VII collagen: the anchoring fibril protein at fault in dystrophic epidermolysis bullosa. Dermatol Clin 28:93-105

Mahoney, My G; Sadowski, Sara; Brennan, Donna et al. (2010) Compound heterozygous desmoplakin mutations result in a phenotype with a combination of myocardial, skin, hair, and enamel abnormalities. J Invest Dermatol 130:968-78

Remington, Jennifer; Wang, Xinyi; Hou, Yingpin et al. (2009) Injection of recombinant human type VII collagen corrects the disease phenotype in a murine model of dystrophic epidermolysis bullosa. Mol Ther 17:26-33

Uitto, Jouni (2009) Progress in heritable skin diseases: translational implications of mutation analysis and prospects of molecular therapies*. Acta Derm Venereol 89:228-35

Lugassy, Jennie; McGrath, John A; Itin, Peter et al. (2008) KRT14 haploinsufficiency results in increased susceptibility of keratinocytes to TNF-alpha-induced apoptosis and causes Naegeli-Franceschetti-Jadassohn syndrome. J Invest Dermatol 128:1517-24

Igoucheva, Olga; Kelly, Aislinn; Uitto, Jouni et al. (2008) Protein therapeutics for junctional epidermolysis bullosa: incorporation of recombinant beta3 chain into laminin 332 in beta3-/- keratinocytes in vitro. J Invest Dermatol 128:1476-86

Nakajima, Koji; Tamai, Katsuto; Yamazaki, Takehiko et al. (2008) Identification of Skn-1n, a splice variant induced by high calcium concentration and specifically expressed in normal human keratinocytes. J Invest Dermatol 128:1336-9

Varki, Roslyn; Sadowski, Sara; Uitto, Jouni et al. (2007) Epidermolysis bullosa. II. Type VII collagen mutations and phenotype-genotype correlations in the dystrophic subtypes. J Med Genet 44:181-92

Nyquist, Gurston G; Mumm, Christina; Grau, Renee et al. (2007) Malignant proliferating pilar tumors arising in KID syndrome: a report of two patients. Am J Med Genet A 143:734-41