Loss of endoplasmic reticulum-associated enzymes in affected brain regions in Huntington's disease and Alzheimer-type dementia.

Abstract

Post-mortem brain samples from patients with either Huntington's disease, Alzheimer-type dementia or appropriate controls were assayed for endoplasmic reticulum enzymes, NADPH-cytochrome c reductase, neutral alpha-glucosidase, inosine diphosphatase, alpha-mannosidase and glucose-6-phosphatase and for Golgi enzymes, fucosyl- and galactosyl-transferases. In Alzheimer-type dementia there was a selective decrease in alpha-glucosidase activity in the temporal cortex. In Huntington's disease there was a selective decrease of putamen alpha-glucosidase and fucosyl-transferase activities. It is suggested that these changes reflect highly specific alterations in glycoprotein synthesis and processing and may contribute to the underlying pathology of these disorders.

• Authors: A J Cross, T J Crow, J A Johnson, J M Dawson, T J Peters
• Journal: Journal of the neurological sciences (J Neurol Sci) Vol. 71 Issue 2-3 Pg. 137-43 (Dec 1985) ISSN: 0022-510X [Print] Netherlands
• PMID: 2935594 (Publication Type: Journal Article)

Chemical References

• NADPH-Ferrihemoprotein Reductase
• alpha-Glucosidases

Topics

• Adult
• Aged
• Alzheimer Disease (enzymology)
• Brain (enzymology)
• Cerebral Cortex (enzymology)
• Endoplasmic Reticulum (enzymology)
• Female
• Humans
• Huntington Disease (enzymology)
• Male
• Middle Aged
• NADPH-Ferrihemoprotein Reductase (analysis)
• Putamen (enzymology)
• alpha-Glucosidases (analysis)