Mutant Huntingtin affects toll-like receptor 4 intracellular trafficking and cytokine production in mast cells.

ABSTRACT:

Background

Huntington's disease (HD) is caused by the expression of a mutated variant of Huntingtin (mHtt), which results in the complex pathology characterized by a defective function of the nervous system and altered inflammatory responses. While the neuronal effects of mHtt expression have been extensively studied, its effects on the physiology of immune cells have not been fully described. Mast cells (MCs) are unique tissue-resident immune cells whose activation has been linked to protective responses against parasites and bacteria, but also to deleterious inflammatory allergic reactions and, recently, to neurodegenerative diseases.

Methods

Bone marrow-derived mast cells (BMMCs) were obtained from wild-type (WT-) and mHtt-expressing (R6/1) mice to evaluate the main activ

SUBMITTER: Perez-Rodriguez MJ 

PROVIDER: S-EPMC7102443 | biostudies-literature | 2020 Mar

REPOSITORIES: biostudies-literature