# IMMUNOREGULATION IN AVIAN SCLERODERMA

> **NIH NIH R01** · UNIVERSITY OF CALIFORNIA AT DAVIS · 1992 · $173,565

## Abstract

UCD line 200 chickens develop an inherited autoimmune fibrotic disease,
several features of which are similar to human progressive systemic
sclerosis (SSc). The most striking features of this disease are mononuclear
cell infiltrate in skin as well as hyperplasia of the media and intimal
destruction of the arterial vessels, and fibrosis. These findings are found
in skin, esophagus, heart, lung, testes, synovia, and other organs. Line
200 birds also develop antinuclear antibodies and antibodies to type II
collagen. Using a detailed panel of monoclonal antibodies to avian
mononuclear cell populations, we have demonstrated that early lesions show
a significant CT4 cell infiltration followed by CT8 cells and an increase
in B cell clusters. We have also developed an extensive panel of monoclonal
antibodies to thymic stroma. These antibodies demonstrate that line 200
chickens have striking deficiencies in the normal thymic subcapsular
architecture coupled with an excessive expression of MHC class II antigens,
particularly in the cortex. In addition, line 200 chickens have a
significant elevation of IL-2 receptor thymic density. These thymic lesions
can be found as early as 18 days in ovo, long before any disease is
manifest. We will build on the strengths of this model and attempt to study
in detail select aspects of T cell differentiation and the role of soluble
factors and accessory molecules in inducing fibroblast and endothelial cell
destruction. In addition, we will use newly developed reagents to determine
why line 200 thymocytes have increased IL-2R levels and whether monoclonal
antibodies to CT4, CT8, B-L or B cells significantly alter disease
expression. Further, because of the significant degree of dermal pathology,
we will study in parallel the immunobiology of lesions by determining
whether there are functional differences in dermal fibroblasts of line 200
chickens compared to controls. Similarly, we will determine whether
mononuclear cells and/or mononuclear cell derived cytokines from line 200
or control birds influence the growth and biosynthetic function of line 200
and control derived fibroblasts. Finally, we will take advantage of the
unique embryogenesis of the chicken and study factors produced by line 200
chickens which promote angiogenesis as well as to determine the basis of
vascular occlusion in affected animals.

## Key facts

- **NIH application ID:** 3157793
- **Project number:** 5R01AR036867-06
- **Recipient organization:** UNIVERSITY OF CALIFORNIA AT DAVIS
- **SAM.gov UEI:** TX2DAGQPENZ5
- **Principal Investigator:** MERRILL E GERSHWIN
- **Activity code:** R01 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 1992
- **Award amount:** $173,565
- **Award type:** 5
- **Project period:** 1987-07-01 → 1993-06-30

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/3157793

## Citation

> US National Institutes of Health, RePORTER application 3157793, IMMUNOREGULATION IN AVIAN SCLERODERMA (5R01AR036867-06). Retrieved via MORVS 2026-10-25 from https://api.morvs.ai/grant/nih/3157793. Licensed CC0.

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