Age is an important independent modifier of SCA3 phenotype severity.

Neuroscience Letters
Shujun JiaoHong Jiang

Abstract

This study aimed to investigate factors modulating spinocerebellar ataxia type 3 (SCA3) phenotype severity besides the expanded CAG repeats (ExpCAG) of ATXN3. Data regarding CAG trinucleotide repeats, age at onset (AO), duration, age, sex, transmitting parent, and scale scores of SCA3 patients were collected. Multiple linear regression analysis was performed to identify influential independent variables. Age, AO, ExpCAG, and duration were considered control variables to analyze the correlation between independent variables and scale scores. Duration, age, and ExpCAG were screened as influential independent variables (P = 0.000). Age had the greatest impact on multiple linear regression models (P<5E-8). ExpCAG and SARA/ICARS/INAS/Barthel index were not correlated (P > 0.05); considering only age as the control, ExpCAG was slightly-to-moderately correlated with all aforementioned scores except INAS (P < 0.05). Age and all scores, except INAS, were positively correlated (P < 0.05); considering duration, AO, or ExpCAG as controls, their correlations did not change significantly. On controlling age, AO was negatively correlated with all scores (P < 0.05), except for the Barthel index (P > 0.05). Furthermore, the interaction model re...Continue Reading

References

Feb 11, 1988·Nucleic Acids Research·S A MillerH F Polesky
Jan 1, 1995·European Journal of Endocrinology·J BertheratJ Epelbaum
Mar 10, 1999·Journal of Neurology, Neurosurgery, and Psychiatry·T KlockgetherJ Dichgans
Jan 28, 2004·Neurogenetics·Anton van Dellen, Anthony J Hannan
Dec 7, 2007·Brain : a Journal of Neurology·Keisuke SuzukiGen Sobue
Jun 3, 2008·Movement Disorders : Official Journal of the Movement Disorder Society·Bernard RavinaMichael P McDermott
Dec 17, 2011·Movement Disorders : Official Journal of the Movement Disorder Society·Adam RosenblattChristopher A Ross
Jul 10, 2012·Brain : a Journal of Neurology·Atsushi HashizumeGen Sobue
Jan 30, 2014·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·H JiangUNKNOWN Chinese HD Network
Jun 29, 2014·Brain : a Journal of Neurology·Sophie Tezenas du MontcelGiovanni Stevanin
Dec 4, 2014·Journal of the Neurological Sciences·Vívian Pedigone CintraWilson Marques
Jul 1, 1991·Multivariate Behavioral Research·S B Green
Mar 25, 2017·Lancet·Layal ChakerRobin P Peeters
Feb 7, 2018·Clinics in Perinatology·Ari J Wassner
Feb 15, 2018·Brain : a Journal of Neurology·Zhao ChenHong Jiang
Mar 20, 2018·Lancet Neurology·Alhassane DialloSophie Tezenas du Montcel
May 11, 2018·Neurology·N Ahmad AzizG Bernhard Landwehrmeyer
Oct 26, 2018·Ageing Research Reviews·Zhao ChenHong Jiang
Mar 21, 2019·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·P WangH Jiang
Jul 18, 2020·Science·Monia BarnatSandrine Humbert

❮ Previous
Next ❯

Citations

Aug 17, 2021·Movement Disorders : Official Journal of the Movement Disorder Society·Jeannette Hübener-SchmidOlaf Riess

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.