Biochemical abnormalities in Huntington's chorea brains

The enzymes glutamic acid decarboxylase, choline acetylase, and succinate dehydrogenase were reduced by 74 to 93 percent in striatum of brains from three patients who died with long-standing Huntington's chorea. Little change was found in acetylcholinesterase or monoamine oxidase activities. In an early case of this disease in a fourth patient, there were no enzymatic changes. A high-molecular-weight soluble protein was present in the striatum of the advanced cases but membrane protein patterns did not differ from those of controls.

[1]  H. Fibiger,et al.  Choline acetylase and glutamic acid decarboxylase in Huntington's chorea , 1973, Neurology.

[2]  J. Wu,et al.  Purification and characterization of glutamate decarboxylase from mouse brain. , 1973, The Journal of biological chemistry.

[3]  D. Wallach,et al.  Electrophoretic analysis of the major polypeptides of the human erythrocyte membrane. , 1971, Biochemistry.

[4]  A. Hamberger,et al.  COMPARATIVE STUDIES ON MITOCHONDRIA ISOLATED FROM NEURON-ENRICHED AND GLIA-ENRICHED FRACTIONS OF RABBIT AND BEEF BRAIN , 1970, The Journal of cell biology.

[5]  K. Weber,et al.  The reliability of molecular weight determinations by dodecyl sulfate-polyacrylamide gel electrophoresis. , 1969, The Journal of biological chemistry.

[6]  R. Mccaman,et al.  Microdctermination of choline acetyltransferase —A comparison of reinecke vs. periodide precipitation , 1969, Journal of neurochemistry.

[7]  R. Mccaman,et al.  Radiomimetric assay of acetylcholinesterase activity in submicrogram amounts of tissue. , 1968, Life sciences.

[8]  R. Mccaman,et al.  MICRODETERMINATION OF CHOLINE ACETYLASE IN NERVOUS TISSUE , 1965, Journal of neurochemistry.

[9]  R. Mccaman,et al.  MICRODETERMINATION OF MONOAMINE OXIDASE AND 5‐HYDROXYTRYPTOPHAN DECARBOXYLASE ACTIVITIES IN NERVOUS TISSUES , 1965, Journal of neurochemistry.

[10]  T. Slater,et al.  STUDIES ON SUCCINATE-TETRAZOLIUM REDUCTASE SYSTEMS. III. POINTS OF COUPLING OF FOUR DIFFERENT TETRAZOLIUM SALTS. , 1963, Biochimica et biophysica acta.

[11]  E. Roberts,et al.  Some properties of L-glutamic decarboxylase in mouse brain. , 1963, Biochemical pharmacology.

[12]  Oliver H. Lowry,et al.  Protein measurement with the Folin phenol reagent. , 1951, The Journal of biological chemistry.

[13]  T. Perry,et al.  Huntington's chorea. Deficiency of gamma-aminobutyric acid in brain. , 1973, The New England journal of medicine.

[14]  W. L. Stahl.  Phospholipase C purification and specificity with respect to individual phospholipids and brain microsomal membrane phospholipids. , 1973, Archives of biochemistry and biophysics.

[15]  S. Hotta,et al.  The effects of aminooxyacetate on the metabolism of glucose and glutamate by homogenates of guinea pig cerebral hemispheres. , 1970, Toxicology and applied pharmacology.