Transgenic minipig model of Huntington's disease exhibiting gradually progressing neurodegeneration

A transgenic minipig model of Huntington's Disease.

BACKGROUND Some promising treatments for Huntington's disease (HD) may require pre-clinical testing in large animals. Minipig is a suitable species because of its large gyrencephalic brain and long lifespan. OBJECTIVE To generate HD transgenic (TgHD) minipigs encoding huntingtin (HTT)1-548 under the control of human HTT promoter. METHODS Transgenesis was achieved by lentiviral infection of ...

Multi-transgenic minipig models exhibiting potential for hepatic insulin resistance and pancreatic apoptosis

There are currently no multi‑transgenic minipig models of diabetes for the regulation of multiple genes involved in its pathogenesis. The foot and mouth disease virus 2A (F2A)‑mediated polycistronic system possesses several advantages, and the present study developed a novel multi‑transgenic minipig model associated with diabetes using this system. The tissue‑specific polycistronic system used ...

Applying Bioinfomatics Strategies in Huntingtons Disease Research

From neuronal inclusions to neurodegeneration: neuropathological investigation of a transgenic mouse model of Huntington's disease.

Huntington's disease (HD) is an inherited progressive neurodegenerative disease caused by the expansion of a polyglutamine repeat sequence within a novel protein. Recent work has shown that abnormal intranuclear inclusions of aggregated mutant protein within neurons is a characteristic feature shared by HD and several other diseases involving glutamine repeat expansion. This suggests that in ea...

Amyloid pathology is associated with progressive monoaminergic neurodegeneration in a transgenic mouse model of Alzheimer's disease.

beta-Amyloid (Abeta) pathology is an essential pathogenic component in Alzheimer's disease (AD). However, the significance of Abeta pathology, including Abeta deposits/oligomers and glial reactions, to neurodegeneration is unclear. In particular, despite the Abeta neurotoxicity indicated by in vitro studies, mouse models with significant Abeta deposition lack robust and progressive loss of fore...