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Endoplasmic reticulum-associated beta-amyloid peptide binding protein (ERAB) is an intracellular polypeptide that binds to beta-amyloid-beta, a neurotoxic peptide that has been implicated in the pathogenesis of Alzheimer's disease. ERAB is a hydroxysteroid dehydrogenase enzyme expressed in normal tissues, but overexpressed in neurons affected in Alzheimer's disease. Overexpression of ERAB in cultured cells enhances the neurotoxic effects of beta-amyloid. Defects in ERAB are the cause of neurological abnormalities including mental and psychomotor retardation.