Skip Navigation
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

National Institute of Environmental Health Sciences

Use this QR code to view the newest version of this document
Use this QR code to view the newest version of this document

Your Environment. Your Health.

Publication Detail

Title: Identification of alpha-fetoprotein as an autoantigen in juvenile Batten disease.

Authors: Castaneda, Julian A; Pearce, David A

Published In Neurobiol Dis, (2008 Jan)

Abstract: Humoral autoimmunity against glutamic acid decarboxylase has been described in juvenile Batten disease patients and in the Cln3(-/-) mouse model. To obtain a more comprehensive understanding of the repertoire of antigens targeted, we examined the reactivity of Cln3(-/-) mouse sera to brain proteins from fetal, postnatal and adult rats. Among the candidate antigens identified was alpha-fetoprotein (AFP), a protein that has altered expression in several nervous system disorders and hepatic malignancies. Moreover, AFP levels were upregulated in the brains and livers of postnatal day 14 Cln3(-/-) animals. Sera from 31 juvenile Batten disease patients revealed the presence of anti-AFP autoantibodies in juvenile Batten disease male patients (12/13) and female patients (8/18). While these findings provide more evidence that autoimmunity is an active component of juvenile Batten disease, the gender-apparent difference evidenced by patients with regard anti-AFP antibodies may underlie variation in progression and clinical manifestations in this disorder.

PubMed ID: 17931875 Exiting the NIEHS site

MeSH Terms: Adolescent; Adult; Animals; Animals, Newborn; Autoantibodies/metabolism; Autoantigens/metabolism*; Child; Disease Models, Animal; Electrophoresis, Gel, Two-Dimensional/methods; Embryo, Mammalian; Female; Gene Expression Regulation, Developmental/physiology; Humans; Liver Function Tests/methods; Liver/metabolism; Male; Membrane Glycoproteins/deficiency; Mice; Mice, Knockout; Molecular Chaperones; Neuronal Ceroid-Lipofuscinoses/immunology*; Neuronal Ceroid-Lipofuscinoses/metabolism*; Sequence Analysis, Protein; Tandem Mass Spectrometry/methods; alpha-Fetoproteins/analysis*; alpha-Fetoproteins/metabolism*

Back
to Top