Open AccessPatient‐derived olfactory mucosa for study of the non‐neuronal contribution to amyotrophic lateral sclerosis pathology
Author(s) -
GarcíaEscudero Vega,
Rosales María,
Muñoz José Luis,
Scola Esteban,
Medina Javier,
Khalique Hena,
Garaulet Guillermo,
Rodriguez Antonio,
Lim Filip
Publication year - 2015
Publication title -
journal of cellular and molecular medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.44
H-Index - 130
eISSN - 1582-4934
pISSN - 1582-1838
DOI - 10.1111/jcmm.12488
Subject(s) - amyotrophic lateral sclerosis , spinal cord , motor neuron , pathology , biology , neuroscience , olfactory mucosa , sod1 , superoxide dismutase , disease , medicine , olfactory system , oxidative stress , endocrinology
Abstract Amyotrophic lateral sclerosis ( ALS ) is a degenerative motor neuron disease which currently has no cure. Research using rodent ALS models transgenic for mutant superoxide dismutase 1 ( SOD 1) has implicated that glial–neuronal interactions play a major role in the destruction of motor neurons, but the generality of this mechanism is not clear as SOD 1 mutations only account for less than 2% of all ALS cases. Recently, this hypothesis was backed up by observation of similar effects using astrocytes derived from post‐mortem spinal cord tissue of ALS patients which did not carry SOD 1 mutations. However, such necropsy samples may not be easy to obtain and may not always yield viable cell cultures. Here, we have analysed olfactory mucosa (OM) cells, which can be easily isolated from living ALS patients. Disease‐specific changes observed when ALS OM cells were co‐cultured with human spinal cord neurons included decreased neuronal viability, aberrant neuronal morphology and altered glial inflammatory responses. Our results show the potential of OM cells as new cell models for ALS .