Basal ganglia in Huntington’s disease

Glass M, Dragunow M, Pauli RLM (2000) The pattern of neurodegenerahon in Huntington s disease a comparative study of cannabinoid, dopamine, adenosine and GABAa receptor alterations in the human basal ganglia in Huntington s disease. Neuroscience 97 505-519... 

Despite the evidence of a decreased endocannabinoid transmission in the basal ganglia in Huntington s disease and the suggestion that CB, receptor activation might play an instrumental... 

Alterations in brain iron metabolism have been reported, resulting in increased iron accumulation in Huntington s disease. This was particularly the case in basal ganglia from patients with HD compared to normal controls. In studies in embryonic stem cells, huntingtin was found to be iron-regulated, essential for the function of normal nuclear and perinuclear organelles and to be involved in the regulation of iron homeostasis. 

Browne SE, Bowhng AC, MacGarvey U, Baik MJ, Berger SC, Muqit MMK, Bird ED, Beal MF (1997) Oxidative damage and metabolic dysfunction in Huntington s disease selective vulnerability of the basal ganglia. Ann Neurol 41 646-653... 

Basal ganglia are a group of subcortical nuclei which are essential for the coordination of movements (so-called extrapyramidal system). They include the caudate nucleus, putamen, globus pallidus, and lenti-form nucleus. Damage of the basal ganglia results in involuntary movements, as are observed in Parkinson s disease and Huntington s chorea. 

Basal ganglia A group of networked structures in the brain which control voluntary movement. Two basal ganglia disorders are Huntington s disease and Parkinson s disease. 

Neurodegenerative changes in basal ganglia as in Parkinson s disease, Huntington s chorea, Wilson s disease... 

Symptomatically, Huntington s disease is characterized by dyskinesias (choreoform movements) which start with the fingers and then spread to the rest of the body. Akinesia also occurs as a result of the basal ganglia dysfunction. However, unlike most types of Parkinson s disease, Huntington s disease develops during early middle age (30-40 years), it is progressive and results in death in approximately 20 years from the start of the symptoms. In the final years of the disease, the patient suffers severe motor impairment, emotional lability and dementia. 

Krammer EB (1980) Anterograde and transsynaptic degeneration en cascade in basal ganglia induced by intrastriatal injection of kainic acid an animal analogue of Huntington s disease. Brain Res 796(1) 209-221. 

Lastres-Becker I, Fezza F, Cebeira M, Bisogno T, Ramos JA, Milone A, Fernandez-Ruiz J, Di Marzo V (2001) Changes in endocannabinoid transmission in the basal ganglia in a rat model of Huntington s disease. Neuroreport 12 2125-2129... 

Lastres-Becker I, Gomez M, De Miguel R, Ramos JA, Fernandez-Ruiz J (2002b) Loss of cannabinoid CBI receptors in the basal ganglia in the late akinetic phase of rats with experimental Huntington s disease. Neurotox Res 4 601-608... 

Lastres-Becker 1, Berrendero F, Lucas JJ, Marli n-Aparicio E, Yamamoto A, Ramos JA, Ferndndez-Ruiz J (2002c) Loss of mRNA levels, binding and activation of GTP-binding proteins for cannabinoid CBI receptors in the basal ganglia of a transgenic model of Huntington s disease. Brain Res 929 236-242... 

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