Huntington's disease is caused by a mutation in the Huntingtin gene (HTT), which appears in adults and features motor, cognitive and psychiatric alterations.
Protein alteration contributes to degeneration of neuronal populations in Huntington s disease
Protein alteration in the family of lamins causes several diseases, known as laminopathies, such as progeria or precocious aging.
A study in which UB researchers have taken part states that alterations in the levels of one of these proteins, lamin B1, contribute to the degeneration of different brain neuronal populations in Huntington s disease. Caused by a mutation in the huntingtin gene, this pathology features involuntary movements, cognitive deficit, and psychiatric disorders, and has no cure yet.
According to the study, published in the journal
EMBO Molecular Medicine, these results open new therapeutic pathways for the treatment of this disease, since research shows pharmacological normalization of levels of lamin B1 improves the cognitive symptoms in a transgenic model of the disease.
From left to right: Carla Castany-Pladevall, Esther Pérez-Navarro and Arantxa Golbano.
From left to right: Rafael Alcalá-Vida and Marta Garcia-Forn.
These images show the nucleus of neurons in the CA1 region of the hippocampus with a marker for lamina B1. It shows the altered morphology of the nuclei of neurons in the R6/1 mouse, a model of Huntington’s disease.
Protein alteration in the family of lamins causes several diseases, known as laminopathies, such as progeria or precocious ageing. A study in which UB researchers have taken part states that alterations in the levels of one of these proteins, lamin B1, contribute to the degeneration of different brain neuronal populations in Huntington’s disease. Caused by a mutation in the huntingtin gen, this pathology features involuntary movements, cognitive deficit and psychiatric disorders, and has no cure yet. According to the study, published in the journal EMBO Molecular Medicine, these results open new therapeutic pathways