Section of Allergy and Immunology

• Ramsay Fuleihan, Associate Research Scientist
  Special Interests: Molecular mechanism and therapy of primary immune deficiency diseases, Development of the immune system
• Ping-xia Zhang, Associate Research Scientist
  Special Interests: Gene therapy of primary immune deficiency diseases

Non-Faculty members (technical, clerical)

• Janet Watson, Administrative assistant

Fellows and other trainees

• Agnieczka Matczuk, 3rd year fellow
• Cheri Lin, 2nd year fellow

Current Grant Support

Name	Title	Funding Agency	Dates
Ramsay Fuleihan	Ontogeny of lymphocyte development	Charles Hood Foundation	6/1/02 – 5/31/03

Honors and Awards for 2001 and 2002

• Ramsay Fuleihan, Editor, Allergy and Immunology Section, Current Opinion of Pediatrics
• Co-chair, Hyper IgM group, Pan American Group for Immune Deficiency
• Consulting Immunologist, Immune Deficiency Foundation
• Co-director, Allergy and Immunology Fellowship Training Program, Yale University School of Medicine and Yale/New Haven Hospital
• Abstract Review Committee, Basic and Clinical Immunology Interest Section, American Academy of Allergy, Asthma, and Immunology
• 11/2-3/2001 Co-director, Northeast National Resident Education Program, American Academy of Allergy, Asthma, and Immunology

Publications for 2001 and 2002:

Original reports:
1. Zhang, P, E Cooper, R Loomis, RA Flavell, and RL Fuleihan. 2001. Expression of a human CD40 ligand transgene in CD40 ligand-deficient mice, Transgenics, 3: 227-236.

2. Bennett, CL, ME Brunkow, F Ramsdell, KC O'Briant, Q Zhu, RL Fuleihan, AO Shigeoka, HD Ochs, and PF Chance. 2001. A rare polyadenylation signal mutation of the FOXP3 gene (AAUAAA-- >AAUGAA) leads to the IPEX syndrome. Immunogenetics 53:435-439.

3. Lobo, FM, P Scholl and RL Fuleihan. 2002. CD40 ligand deficient T cells in carriers of X-linked hyper IgM have intrinsic priming capability. J. Immunol., 168: 1473-1478.

4. Bogue, CW, P-X Zhang, J McGrath, HC Jacobs, and RL Fuleihan. 2003. Impaired B cell development and function in mice with a targeted disruption of the homeobox gene Hex, Proc. Natl. Acad. Sci. USA,100:556-61.

Reviews and Chapters:
1. Fuleihan, RL. 2001. The Hyper IgM Syndrome. Current Allergy Reports, 1: 445-450

2. Fuleihan, RL. 2001. Hyper IgM Syndrome: The other side of the coin. Curr. Opin. Pediat., 13: 528-532.

3. Fuleihan, RL. 2002. Editorial Review: The hygiene hypothesis and atopic disease. Curr. Opin. Pediat., 14: 676-677.

4. Fuleihan, RL. Antibody deficiency syndrome. In Current Therapy in Allergy, Immunology, and Rheumatology, 2002, Sixth Edition, Lichtenstein and Geha, eds., Mosby, St. Louis, In Press.

Research Interests and Activity
The main interest of my laboratory is to delineate the molecular and cellular basis of primary immune deficiency disease and their therapy. We, as well as others, discovered the genetic basis of the X-linked hyper IgM syndrome to be defects in the CD40 ligand gene. X-linked hyper IgM is an immune deficiency that is characterized by the inability to generate antibody because of the failure of interaction between T cells and B cells. Affected patients suffer from recurrent sino-pulmonary infections as well as opportunistic infections and have a higher susceptibility to cancer. Therefore, we have been using a mouse model of X-linked hyper IgM to study gene therapy of this disease. We have also been investigating the regulation of CD40 ligand expression because it is critical for its function. The results of these studies may lead to a genetic therapy approach to X-linked hyper IgM, which may also be applicable to other genetic diseases. In addition, we are interested in the development of the immune system. In collaboration with Clifford Bogue at the YCHRC, we have demonstrated a critical role for the homeobox gene Hex in B cell development and function and we are currently determining the mechanism by which Hex affects B cell development. The results of this work may identify other factors that are necessary for B cell development, which may help identify causes of agammaglobulinemia in children.