Selective Sparing of a Class of Striatal Neurons in Huntington's Disease

• Published in: Science (American Association for the Advancement of Science) Vol. 230; no. 4725; pp. 561 - 563
• Main Authors: Ferrante, R. J., Kowali, N. W., Beal, M. F., Richardson, E. P., Bird, E. D., Martin, J. B.
• Format: Journal Article
• Language: English
• Published: Washington, DC The American Association for the Advancement of Science 01.11.1985; American Association for the Advancement of Science
• Subjects: Adult; Aged; Biochemistry; Biological and medical sciences; Caudate nucleus; Caudate Nucleus - enzymology; Caudate Nucleus - pathology; Corpus Striatum - enzymology; Corpus Striatum - pathology; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Enzymes; Epithelial cells; Gels; Humans; Huntington disease; Huntington Disease - enzymology; Huntington Disease - pathology; Huntington's chorea; Huntington's disease; Medical sciences; Middle Aged; NADPH Dehydrogenase - analysis; Nerve Tissue Proteins - analysis; Neurology; Neurons; Neurons - enzymology; Neurons - pathology; Neuropeptide Y; Neuropeptides; Perfusion; Physiological aspects; Trajectory control; Human; Pathology; Nervous system diseases; Neuron; Huntington disease; Heredodegeneration; Brain (Vertebrata); Corpus striatum
• ISSN: 0036-8075; 1095-9203
• DOI: 10.1126/science.2931802

A distinct subpopulation of striatal aspiny neurons, containing the enzyme nicotinamide adenine dinucleotide phosphate diaphorase, is preserved in the caudate nucleus in Huntington's disease. Biochemical assays confirmed a significant increase in the activity of this enzyme in both the caudate nucleus and putamen in postmortem brain tissue from patients with this disease. The resistance of these neurons suggests that the gene defect in Huntington's disease may be modifiable by the local biochemical environment. This finding may provide insight into the nature of the genetically programmed cell death that is a characteristic of the disease.