An autoantibody inhibitory to glutamic acid decarboxylase in the neurodegenerative disorder Batten disease

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An autoantibody inhibitory to glutamic acid decarboxylase in the neurodegenerative disorder Batten disease.

Mutations in the CLN3 gene are responsible for the neurodegenerative disorder Batten disease; however, the molecular basis of this disease remains unknown. In studying a mouse model for Batten disease, we report the presence of an autoantibody to glutamic acid decarboxylase (GAD65) in cln3-knockout mice serum that associates with brain tissue but is not present in sera or brain of normal mice. ...

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Monoclonal antibodies to glutamic acid decarboxylase.

Five monoclonal antibodies that recognize chicken brain glutamic acid decarboxylase (GAD) have been selected and designated GAD-1 to -5. GAD-1 to -5 were selected on the basis of their ability to immunoprecipitate active GAD from crude brain extracts. GAD-1 recognizes an epitope that is conserved in many vertebrates; the epitope recognized by GAD-5 is restricted to the chicken. Radioimmunoassay...

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Antibodies to Inhibitory Synaptic Proteins in Neurological Syndromes Associated with Glutamic Acid Decarboxylase Autoimmunity

Antibodies to glutamic acid decarboxylase (GAD-ab) associate to different neurological syndromes. It is unknown if the diversity in syndrome association represents epitopes in different immunodominant domains or co-existence of antibodies to other proteins of the inhibitory synapsis. We examined the serum and CSF of 106 patients with anti-GAD related syndromes (39 cerebellar ataxia, 32 stiff-pe...

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Glutamic acid decarboxylase in Rhodotorula glutinis.

1. The metabolism of 131I-labelled thyroglobulin from four mammalian species has been studied. 2. The rat, dog and monkey rapidly metabolize the homologous protein, with the formation of iodide, iodotyrosines and thyroxine. Rabbit thyroglobulin disappears slowly from the plasma of the rabbit after intravenous injection, and no metabolites have so far been detected in the blood. 3. The developme...

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ژورنال

عنوان ژورنال: Human Molecular Genetics

سال: 2002

ISSN: 1460-2083

DOI: 10.1093/hmg/11.12.1421