Accordingly, post-mortem studies of HD patient brain tissue have shown significant neuropathology including mutant huntingtin protein (mHTT) aggregate formation, profound neuronal death and gliosis 3– 5. When the glutamine stretch in the N-terminus of huntingtin protein (HTT) exceeds approximately 40 repeats, HTT misfolds and sets off a toxic sequence of events inside the cell including transcriptional dysregulation, mitochondrial dysfunction, calcium signaling disruption and altered neurotransmission, including glutamatergic and dopaminergic dysregulation 2. Huntington’s disease (HD) is a genetic, progressive neurodegenerative disorder caused by an expanded CAG repeat in exon 1 of the HTT gene 1. This novel model will aid in the development of disease biomarkers and therapeutic strategies for this devastating disorder. Multimodal imaging revealed circuit-wide white and gray matter degenerative processes in several key brain regions affected in HD. Compared to controls, mHTT-treated macaques developed progressive working memory decline and motor impairment. We queried the disruption of cortico-basal ganglia circuitry for 20-months post-surgery using a variety of behavioral and imaging readouts. This novel modeling strategy results in robust expression of mutant huntingtin protein (mHTT) in the injected brain regions, as well as dozens of other cortical and subcortical brain regions that are also affected in human HD patients. Our findings indicate an interdependence between CAG repeats number within the IT-15 gene, the course of the disease and the clinical status of HD patients.We created a new nonhuman primate model of the genetic neurodegenerative disorder, Huntington’s disease (HD), by injecting a mixture of recombinant adeno-associated viral vectors, serotypes AAV2 and AAV2.retro, each expressing a fragment of human mutant HTT ( mHTT) into the caudate and putamen of adult rhesus macaques. Negative significant correlation between time from the onset and MUHDRS score (r = -0.95 p < 0.05) and negative correlation between summarised: time from the onset and CAG number on the one site and MUHDRS on the other (p = -0.91) were found, as well. A negative significant correlation was found between the CAG repeats number and the disease onset age (r = -0.67 p < 0.05) and MUHDRS score (r = 0.75 p < 0.05), as well. The number of CAG repeats in examination of the IT-15 gene was determined by polymerase chain reaction (PCR) and separation of radioisotope labelled PCR product against DNA size marker in polyacrylamide gel. Modified UHDRS (MUHDRS) was applied for the estimation of patients' clinical status. The aim of the present study was to search for possible correlation between the age of the onset of HD, time from the onset, clinical status of the patients, and CAG repeats number. The main symptoms consist of choraetic movements, dementia, and characteropathy.

Huntington's disease (HD) is a neurological condition of progressive course that results from abnormally increased number of CAG repeats within IT-15 gene, coding for huntington.

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