Specific Dystrophins Selectively Associate with Inhibitory and Excitatory Synapses of the Mouse Cerebellum and their Loss Alters Expression of P2X7 Purinoceptors and Pro-Inflammatory Mediators.

Cellular and Molecular Neurobiology
Torquil JacksonJerome D Swinny

Abstract

Duchenne muscular dystrophy (DMD) patients, having mutations of the DMD gene, present with a range of neuropsychiatric disorders, in addition to the quintessential muscle pathology. The neurobiological basis remains poorly understood because the contributions of different DMD gene products (dystrophins) to the different neural networks underlying such symptoms are yet to be fully characterised. While full-length dystrophin clusters in inhibitory synapses, with inhibitory neurotransmitter receptors, the precise subcellular expression of truncated DMD gene products with excitatory synapses remains unresolved. Furthermore, inflammation, involving P2X purinoceptor 7 (P2RX7) accompanies DMD muscle pathology, yet any association with brain dystrophins is yet to be established. The aim of this study was to investigate the comparative expression of different dystrophins, alongside ionotropic glutamate receptors and P2RX7s, within the cerebellar circuitry known to express different dystrophin isoforms. Immunoreactivity for truncated DMD gene products was targeted to Purkinje cell (PC) distal dendrites adjacent to, or overlapping with, signal for GluA1, GluA4, GluN2A, and GluD2 receptor subunits. P2X7R immunoreactivity was located in Ber...Continue Reading

References

Jun 15, 1992·Proceedings of the National Academy of Sciences of the United States of America·D LederfeinU Nudel
Nov 1, 1992·The Journal of Cell Biology·D HouzelsteinM E Buckingham
Mar 16, 1992·Neuroscience Letters·J Huard, J P Tremblay
Feb 1, 1984·Proceedings of the National Academy of Sciences of the United States of America·G BulfieldK J Moore
May 1, 1995·Human Molecular Genetics·V N D'SouzaP N Ray
Mar 1, 1995·Human Molecular Genetics·H G LidovL M Kunkel
May 1, 1993·Nature Genetics·T J ByersL M Kunkel
Sep 1, 1995·Human Molecular Genetics·R C AustinP N Ray
Jun 17, 1998·Developmental Dynamics : an Official Publication of the American Association of Anatomists·K Wertz, E M Füchtbauer
Dec 14, 1999·The European Journal of Neuroscience·I KnueselJ M Fritschy
Oct 4, 2000·The Journal of Biological Chemistry·M SolleC A Gabel
Jul 21, 2001·Developmental Medicine and Child Neurology·S CottonK M Greenwood
Oct 1, 1961·Experimental Neurology·I L MIALE, R L SIDMAN
Jan 28, 2005·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Jun-ichi TanakaRyuichi Shigemoto
Jan 26, 2006·Brain : a Journal of Neurology·Jeremy D Schmahmann, David Caplan
May 20, 2006·Cellular and Molecular Life Sciences : CMLS·T Haenggi, J-M Fritschy
Feb 23, 2007·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Miwako Masugi-TokitaRyuichi Shigemoto
Nov 6, 2007·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Takeshi UemuraMasayoshi Mishina
Feb 5, 2008·Neurochemistry International·David LeónM A Teresa Miras-Portugal
Feb 8, 2008·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Wataru KakegawaMichisuke Yuzaki
Sep 4, 2008·Journal of the International Neuropsychological Society : JINS·Shana E CyrulnikVeronica J Hinton
Sep 12, 2008·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Andrea Lorincz, Zoltan Nusser
Mar 27, 2009·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Mastura MonifDavid A Williams
Sep 29, 2009·Expert Reviews in Molecular Medicine·Dorianna Sandonà, Romeo Betto
Aug 19, 2010·Pharmacological Reviews·Stephen F TraynelisRay Dingledine
Nov 23, 2011·The European Journal of Neuroscience·Masahiko Watanabe, Masanobu Kano
Nov 23, 2011·Molecular Neurobiology·Ramin TadayoniBulmaro Cisneros
Apr 12, 2012·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·Maria Barberà-CremadesPablo Pelegrín
Apr 13, 2012·Neurology·L De PasqualeF De Benedetti
Apr 28, 2012·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Mark D EyreZoltan Nusser
May 26, 2012·Trends in Neurosciences·Adrian WaiteDerek J Blake

❮ 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.

Related Papers

Cellular Oncology (Dordrecht)
Leanne JonesKaren Anthony
Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
Sonia Messina, Gian Luca Vita
© 2021 Meta ULC. All rights reserved