# PATHOPHYSIOLOGY OF EXPERIMENTALLY INDUCED PEMPHIGUS

> **NIH NIH R01** · JOHNS HOPKINS UNIVERSITY · 1989 · $53,251

## Abstract

Over the last three years, our laboratory has been systematically defining
pathogenetic mechanisms that are operative in the autoimmune cutaneous
disease pemphigus.  The animal model we had developed, employing passive
transfer of human autoantibodies in neonatal mice, has allowed us to define
these mechanisms in vivo.  It is our intention to extend these studies to
define the role of plasminogen activator in the production of lesions in
vivo, to examine the potential importance of other proteinases by using
proteinase inhibitors of various specificities to inhibit acantholysis, and
to examine the possible therapeutic effect of drugs that interfere with
cell surface-cytoskeleton interactions and internalization.  A longterm
goal is to expand the current model and attempt to produce disease in these
animals by immunization with antigen.  This would allow us to examine many
more aspects of this complex autoimmune disease such as immune regulation
by idiotypic and anti-idiotypic networks.

Bullous pemphigoid (BP) is another cutaneous blistering disease in which
autoantibodies are present.  We have recently shown that antibodies from BP
patients bind specifically in the area of the cytoplasmic attachment plaque
of the epidermal basal cell hemidesmosome.  We propose a series of studies
to define if there are distinct populations of autoantibodies that bind to
intracellular and/or extracellular BP antigens, if the different
populations of antibodies are complement fixing, and if extracellular
antigen is present in skin only from certain areas of the body.  Once these
questions are answered, we will examine the pathogenicity of these defined
autoantibodies in vivo in mice and in the rabbit cornea.  It is also
possible that factors that permeabilize the basel cell membrane such as
trauma and UV light are necessary to initiate cutaneous lesions, and these
autoantibodies can then bind the cytoplasmic antigen, activate complement
and propagate the lesion.  This series of studies will help us understand
the pathophysiology of this complex blistering disease in vivo.

## Key facts

- **NIH application ID:** 3156322
- **Project number:** 5R01AR032490-07
- **Recipient organization:** JOHNS HOPKINS UNIVERSITY
- **SAM.gov UEI:** FTMTDMBR29C7
- **Principal Investigator:** GRANT J ANHALT
- **Activity code:** R01 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 1989
- **Award amount:** $53,251
- **Award type:** 5
- **Project period:** 1983-08-01 → 1991-07-31

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/3156322

## Citation

> US National Institutes of Health, RePORTER application 3156322, PATHOPHYSIOLOGY OF EXPERIMENTALLY INDUCED PEMPHIGUS (5R01AR032490-07). Retrieved via MORVS 2026-10-25 from https://api.morvs.ai/grant/nih/3156322. Licensed CC0.

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