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. ...

Application of the potent tumor-promoting agent 12-Otetradecanoylphorbol-13-acetate (TPA) to mouse skin leads to a rapid and transient induction of epidermal ornithine decarboxylase (EC 4.1.1.17) activity. Several lines of evidence, including the finding that /3-retinoic acid inhibits both phorbol ester-induced ornithine decarboxyl ase activity and the formation of skin papillomas, suggest that...

Cell growth and differentiation are influenced by intercellular contact, suggesting that cell adhesion molecules may be instrumental in triggering these events. F9 embryonal carcinoma cells are an ideal system in which to examine the function of cell adhesion molecules in growth and differentiation, since the relevant cell adhesion molecules and differentiation markers are well defined. Interce...

*Peripheral neutrophils were supported pretransplant by recombinant granulocyte colony stimulating factor. Post-transplant parameters were obtained at 18 months (FBC and T-cell indices, lung function) or 21 months post-HSCT (B cell and antibody indices). Post-HSCT antibody indices were measured during concomitant subcutaneous immunoglobulin supplementation. No other autoantibodies were detected...

We report the case of a 59-year-old woman who developed rapid-onset type 1 diabetes associated with a marked increase in anti-glutamic acid decarboxylase antibody titer (317.5 U/ml), mild increase in HbA1c level (6.8%), diabetic ketoacidosis, and cytomegalovirus enterocolitis. She was a heterozygote for HLA class II DRB1*0901-DQA1*03-DQB1*0303, and she had HLA class I A24, which may have contri...

We describe a patient who developed significant cognitive decline with profound amnesia following non-dominant temporal lobectomy for refractory seizures, in whom the original suspicion of structural pathology was revised following the discovery of clinical and neuropathological markers of inflammation, neuropsychological evidence of bilateral involvement, and high titres of antibodies directed...