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Related Experiment Video

Updated: Jul 19, 2026

Detection of Disease-associated α-synuclein by Enhanced ELISA in the Brain of Transgenic Mice Overexpressing Human A53T Mutated α-synuclein

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Five cases of alpha chain disease.

W F Doe, K Henry, J R Hobbs

|December 1, 1972

Summary

Alpha chain disease, a rare disorder, involves intestinal inflammation and malabsorption. This study identifies alpha chain fragments in patients and demonstrates treatment success with chemotherapy and steroids.

Area of Science:

Immunology
Gastroenterology
Oncology

Background:

Alpha chain disease is a rare disorder characterized by plasma cell infiltration of the small intestine.
Patients often present with malabsorption symptoms and clubbing.
The disease involves the abnormal production of immunoglobulin A (IgA) heavy chains.

Purpose of the Study:

To describe the clinical and pathological features of alpha chain disease.
To identify the presence and location of alpha chain fragments.
To evaluate treatment outcomes for patients with alpha chain disease.

Main Methods:

Clinical case series of five patients with alpha chain disease.
Histopathological examination of small intestinal biopsies.

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Immunological assays to detect alpha chain fragments in various bodily fluids (serum, urine, jejunal fluid, saliva).

Tissue culture of jejunal biopsy to assess alpha chain synthesis.

Development of a novel immunoselection technique for alpha chain identification.

Main Results:

Patients exhibited characteristic plasma cell infiltrates in the small intestine, with some cases showing spread to bone marrow, peripheral blood, and nasopharyngeal lymphoid tissue.
Alpha chain fragments were detected in serum, urine, jejunal fluid, and saliva of affected patients.
One patient's jejunal biopsy demonstrated in vitro synthesis of free alpha chain.
A new, simple immunoselection technique for identifying free alpha chain was successfully developed.
Two patients achieved significant clinical remission with intermittent cytotoxic and steroid therapy, and a third with cytotoxic therapy and tetracycline.

Conclusions:

Alpha chain disease is a distinct entity with characteristic gastrointestinal and immunological findings.
The detection of alpha chain fragments is a key diagnostic marker.
Combination therapy involving cytotoxic agents, steroids, and antibiotics shows promise for managing alpha chain disease.
The developed immunoselection technique offers a valuable tool for diagnosing alpha chain disease.