title: Modulation at age of onset in tunisian huntington disease patients: implication of new modifier genes.
abstract: Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder. The causative mutation is an expansion of more than 36 CAG repeats in the first exon of IT15 gene. Many studies have shown that the IT15 interacts with several modifier genes to regulate the age at onset (AO) of HD. Our study aims to investigate the implication of CAG expansion and 9 modifiers in the age at onset variance of 15 HD Tunisian patients and to establish the correlation between these modifiers genes and the AO of this disease. Despite the small number of studied patients, this report consists of the first North African study in Huntington disease patients. Our results approve a specific effect of modifiers genes in each population.
authors.affiliation: Department of Cytogenetics and Reproductive Biology, Farhat HACHED Hospital, Ibn El Jazzar Road, 4000 Sousse, Tunisia.
authors.forename: Dorra
authors.initials: D
authors.lastname: Hmida-Ben Brahim
authors.affiliation: Department of Cytogenetics and Reproductive Biology, Farhat HACHED Hospital, Ibn El Jazzar Road, 4000 Sousse, Tunisia.
authors.forename: Marwa
authors.initials: M
authors.lastname: Chourabi
authors.affiliation: Department of Neurology, Sahloul Hospital, Sahloul Road, 4054 Sousse, Tunisia.
authors.forename: Sana
authors.initials: S
authors.lastname: Ben Amor
authors.affiliation: Department of Epidemiology and Medical Statistics, Farhat HACHED Hospital, Ibn El Jazzar Road, 4000 Sousse, Tunisia.
authors.forename: Imed
authors.initials: I
authors.lastname: Harrabi
authors.affiliation: Department of Cytogenetics and Reproductive Biology, Farhat HACHED Hospital, Ibn El Jazzar Road, 4000 Sousse, Tunisia.
authors.forename: Saoussen
authors.initials: S
authors.lastname: Trabelsi
authors.affiliation: Department of Cytogenetics and Reproductive Biology, Farhat HACHED Hospital, Ibn El Jazzar Road, 4000 Sousse, Tunisia.
authors.forename: Marwa
authors.initials: M
authors.lastname: Haddaji-Mastouri
authors.affiliation: Department of Cytogenetics and Reproductive Biology, Farhat HACHED Hospital, Ibn El Jazzar Road, 4000 Sousse, Tunisia.
authors.forename: Moez
authors.initials: M
authors.lastname: Gribaa
authors.affiliation: Department of Cytogenetics and Reproductive Biology, Farhat HACHED Hospital, Ibn El Jazzar Road, 4000 Sousse, Tunisia.
authors.forename: Sihem
authors.initials: S
authors.lastname: Sassi
authors.affiliation: Department of Cytogenetics and Reproductive Biology, Farhat HACHED Hospital, Ibn El Jazzar Road, 4000 Sousse, Tunisia.
authors.forename: Fatma Ezzahra
authors.initials: FE
authors.lastname: Gahbiche
authors.affiliation: Department of Neurology, Sahloul Hospital, Sahloul Road, 4054 Sousse, Tunisia.
authors.forename: Turkia
authors.initials: T
authors.lastname: Lamouchi
authors.affiliation: Department of Cytogenetics and Reproductive Biology, Farhat HACHED Hospital, Ibn El Jazzar Road, 4000 Sousse, Tunisia.
authors.forename: Soumaya
authors.initials: S
authors.lastname: Mougou-Zereli
authors.affiliation: Department of Neurology, Sahloul Hospital, Sahloul Road, 4054 Sousse, Tunisia.
authors.forename: Sofiane
authors.initials: S
authors.lastname: Ben Ammou
authors.affiliation: Department of Cytogenetics and Reproductive Biology, Farhat HACHED Hospital, Ibn El Jazzar Road, 4000 Sousse, Tunisia.
authors.forename: Ali
authors.initials: A
authors.lastname: Saad