Are neuronal intranuclear inclusions the common neuropathology of triplet-repeat disorders with polyglutamine-repeat expansions?

Lancet
S W DaviesG P Bates

Abstract

Neuronal intranuclear inclusions have been found in the brain of a transgenic mouse model of Huntington's disease and in necropsy brain tissue of patients with Huntington's disease. We suggest that neuronal intranuclear inclusions are the common neuropathology for all inherited diseases caused by expansion of polyglutamine repeats. We also suggest that patients with a pathological diagnosis of neuronal intranuclear hyaline inclusion disease may also have polyglutamine repeat expansions.

References

Jun 1, 1986·Neurology·D Munoz-Garcia, S K Ludwin
Jun 1, 1993·Nature Genetics·M SchallingD E Housman
Aug 1, 1997·Nature Genetics·N Heintz, H Zoghbi
Jan 1, 1997·Human Molecular Genetics·G P BatesS W Davies

❮ Previous
Next ❯

Citations

Jun 8, 2000·Muscle & Nerve·A P Lieberman, K H Fischbeck
Aug 12, 1999·Annals of Neurology·A P LiebermanK H Fischbeck
Dec 10, 1999·Annals of Neurology·S KuemmerleR J Ferrante
Sep 29, 2000·Movement Disorders : Official Journal of the Movement Disorder Society·J D O'SullivanA J Lees
Jun 25, 2013·Molecules and Cells·Tiffany W Todd, Janghoo Lim
Jul 20, 2002·Journal of the Neurological Sciences·E MuñozI Ferrer
Nov 6, 1998·Journal of the Neurological Sciences·T E KimberG Suthers
May 12, 2001·Mechanisms of Ageing and Development·D A Butterfield, J Kanski
Aug 15, 2000·Trends in Neurosciences·J H Cha
Feb 22, 2001·Trends in Neurosciences·E CattaneoS Sipione
May 14, 1999·Trends in Neurosciences·P B Tran, R J Miller
Mar 27, 2003·Progress in Neuro-psychopharmacology & Biological Psychiatry·Ryoichi IshitaniNobuo Katsube
Nov 19, 2002·Neuroscience Letters·Johannes SchieferChristoph M Kosinski
Nov 24, 2001·Brain Research Bulletin·M RimoldiV Zimarino
Nov 13, 1998·Current Opinion in Neurobiology·M GoedertS W Davies
Oct 6, 1999·Current Opinion in Neurobiology·H Y Zoghbi, H T Orr
Mar 14, 2013·Nature Communications·Christoph J O KaiserKlaus Richter
Apr 12, 2001·Neuropathology and Applied Neurobiology·K A Sieradzan, D M Mann
Jul 27, 1999·Neuropathology and Applied Neurobiology·M Tolnay, A Probst
Jun 7, 2000·Neuropathology and Applied Neurobiology·J Woulfe, D Munoz
Jan 15, 2004·Neuropathology : Official Journal of the Japanese Society of Neuropathology·Junko Takahashi-Fujigasaki
Feb 12, 2010·Human Molecular Genetics·Jill R CrittendenAnn M Graybiel
Jun 9, 2001·Current Opinion in Obstetrics & Gynecology·C J Loy, E L Yong
Aug 6, 1999·Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences·S W DaviesG P Bates
Aug 6, 1999·Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences·J H ChaA B Young
Aug 6, 1999·Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences·J M OrdwayP J Detloff
Mar 29, 2011·Cold Spring Harbor Perspectives in Biology·Steven Finkbeiner
Feb 15, 2000·Annals of the New York Academy of Sciences·Y NagaiJ R Burke
Oct 12, 1999·Brain Pathology·D W DicksonS H Yen
Nov 10, 2001·Annual Review of Genomics and Human Genetics·C J Cummings, H Y Zoghbi
Jun 9, 2000·Annual Review of Neuroscience·H Y Zoghbi, H T Orr
Jun 1, 2000·Proceedings of the National Academy of Sciences of the United States of America·J A BibbP Greengard
Mar 4, 2000·Proceedings of the National Academy of Sciences of the United States of America·S Krobitsch, S Lindquist
Nov 6, 1998·Brain Pathology·G P BatesS W Davies
Mar 31, 1999·Proceedings of the National Academy of Sciences of the United States of America·E J ParkJ D Chen
Jul 12, 2002·Human Mutation·Damian C Crowther
Dec 16, 1998·The Journal of Cell Biology·M K PerezR N Pittman
Apr 12, 2002·FEBS Letters·Wanjoo ChunGail V W Johnson

❮ 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.