Huntington’s disease neural degeneration, natural
remedy or help
November 23 2015 by Ray Sahelian, M.D.
Huntingtons disease, along with Alzheimer's and Parkinson's, belong to the family of neurodegenerative diseases caused by protein misfolding. The incurable disease is hereditary and has a prevalence of 1 in every 15,000 people. In Germany, about 8,000 cases are currently known, in the UK the figure is reported to be about 5,000, and in the US the figure is about 30,000.
Transl Psychiatry. 2015. N-Acetylcysteine improves mitochondrial function and ameliorates behavioral deficits in the R6/1 mouse model of Huntington's disease. Huntington's disease (HD) is a neurodegenerative disorder, involving psychiatric, cognitive and motor symptoms, caused by a CAG-repeat expansion encoding an extended polyglutamine tract in the huntingtin protein. Oxidative stress and excitotoxicity have previously been implicated in the pathogenesis of HD. We hypothesized that N-acetylcysteine (NAC) may reduce both excitotoxicity and oxidative stress through its actions on glutamate reuptake and antioxidant capacity. The R6/1 transgenic mouse model of HD was used to investigate the effects of NAC on HD pathology. It was found that chronic NAC administration delayed the onset and progression of motor deficits, while having an antidepressant-like effect on both R6/1 and wild-type mice. A deficit in the astrocytic glutamate transporter protein, GLT-1, was found in R6/1 mice. However, this deficit was not ameliorated by NAC, implying that the therapeutic effect of NAC is not due to rescue of the GLT-1 deficit and associated glutamate-induced excitotoxicity. Assessment of mitochondrial function in the striatum and cortex revealed that R6/1 mice show reduced mitochondrial respiratory capacity specific to the striatum. This deficit was rescued by chronic treatment with NAC. There was a selective increase in markers of oxidative damage in mitochondria, which was rescued by NAC. In conclusion, NAC is able to delay the onset of motor deficits in the R6/1 model of Huntington's disease and it may do so by ameliorating mitochondrial dysfunction. Thus, NAC shows promise as a potential therapeutic agent in HD. Furthermore, our data suggest that NAC may also have broader antidepressant efficacy.
Green Tea for Huntington's Disease
The green tea extract, epigallocatechin gallate ( EGCG ), may slow the accumulation of proteins that cause Huntington’s disease. The new research, led by Professor Erich Wanker from the Max Delbrück Center for Molecular Medicine Berlin-Buch (MDC), looked at the effect on EGCG on the aggregation of these mutant proteins in vitro, and found that the green tea extract could interfere with the very early events of this process. The research is published in the September issue journal Human Molecular Genetics (Vol. 15, pp. 2743-2751). Professor Wanker's group used laboratory-based experiments to show that the green tea extract inhibited misfolding of the huntingtin protein in vitro, as well as using fruit flies genetically modified to over-express the mutant protein. The latter experiments showed that the degeneration of the flies' photoreceptor and motor function improved. Further research is needed, particular to discern if the bioactive doses needed to produce these protective effects could be obtained from drinking green tea, or by taking supplements.
No benefit from CoQ10
Comprehensive behavioral testing in the R6/2 mouse model of Huntington's disease shows no benefit from CoQ10 or minocycline.
PLoS One. 2010; Menalled LB, Patry M, Ragland N, Lowden PA, Goodman J, Minnich J, Zahasky B, Park L, Leeds J, Howland D, Signer E, Tobin AJ. PsychoGenics Inc., Tarrytown, New York, United States of America. liliana.
Previous studies of the effects of coenzyme Q10 and minocycline on mouse models of Huntington's disease have produced conflicting results regarding their efficacy in behavioral tests. Using our recently published best practices for husbandry and testing for mouse models of Huntington's disease, we report that neither coenzyme Q10 nor minocycline had significant beneficial effects on measures of motor function, general health (open field, rotarod, grip strength, rearing-climbing, body weight and survival) in the R6/2 mouse model. The higher doses of minocycline, on the contrary, reduced survival. We were thus unable to confirm the previously reported benefits for these two drugs.
Huntington's Disease Symptoms
The disease is characterize by jerky, uncontrolled movements, an unsteady gait and grimaces leading to its original common name of Huntington's chorea (from the ancient Greek for “dance”).
JAMA Neurol. 2013 Dec. Natural history of Huntington
disease. Total motor score worsened by 3.0 points per year and chorea worsened
by 0.3 point per year. Cognition declined by 0.7 point (95% CI, 0.6-0.8) per
year on the Mini-Mental State Examination. Behavior, as measured by the product
of frequency and severity score on the Unified Huntington's Disease Rating
Scale, worsened by 0.6 point per year (95% CI, 0.0-1.2). Total functional
capacity declined by 0.6 point per year (95% CI, 0.5-0.7). Compared with
controls, baseline body mass index was lower in those with Huntington disease
(25.8 vs 28.8; P < .001), and average pulse was higher . Over 3 years, the
cardinal features of Huntington disease all declined in a monotonic manner.
These data quantify the natural history of the disease and may inform the design
of trials aimed at reducing its burden.
Huntington’s disease Gene
In 1993, scientists discovered the gene that encodes the mutant protein, the so-called huntingtin protein. A mutation in this protein results in elongation of parts of the protein called polyglutamine chains, which cause the overall huntingtin protein to lose its normal structure. These mutant proteins can not be disposed of by the body and accumulate in the brain of sufferers, eventually being toxic to the nerve cells in the brain.
There is not a lot of information available to me on my Huntington disease. I have been taking Gingko Biloba & 5HTP 50mg's - these have helped me. I am just wondering if there are anything other supplements I should know about.
A. Only green tea or egcg that we know of thus far.