CHD2009

Factor H-related diseases and animal models

Plan of session:

1) RECOMMENDED READING PRIOR TO SESSION:

Students to have read the following two manuscripts in order that they understand the background of factor H and human disease

Translational mini-review series on complement factor H: genetics and disease associations of human complement factor H.

de Córdoba SR, de Jorge EG.

Clin Exp Immunol. 2008 Jan;151(1):1-13.

weblink to article

Heinen S, Hartmann A, Lauer N, Wiehl U, Dahse HM, Schirmer S, Gropp K, Enghardt T, Wallich R, Halbich S, Mihlan M, Schlotzer-Schrehardt U, Zipfel PF, Skerka C.

Blood. 2009 Jun 15.

weblink to article

2) SESSION OVERVIEW:

Introduction: advantages and limitations of in vitro and of animal models for complement research

5 minutes PZ

Overview of the animal models of FH-related disease 10 minutes MP

FH-deficient animals: Pig, Mouse
FH-transgenic animals

Interactive discussion 60 minutes MP / PZ

What have we learnt from these models?
What are the limitations of existing animal models of FH-related disease?
What models do we need now? – e.g. CFHR, gain of function and autoantibody models
CFHR1 is a complement regulator: What does this tell about the role of CFHR1 in kidney diseases (Hemolytic Uremic Syndrome and in age related macular degeneration)