Curcumin therapy in a Plp1 transgenic mouse model of Pelizaeus-Merzbacher disease

Annals of Clinical and Translational Neurology
Dirk B EpplenMichael W Sereda

Abstract

Pelizaeus-Merzbacher disease (PMD) is a progressive and lethal leukodystrophy caused by mutations affecting the proteolipid protein (PLP1) gene. The most common cause of PMD is a duplication of PLP1 and at present there is no curative therapy available. By using transgenic mice carrying additional copies of Plp1, we investigated whether curcumin diet ameliorates PMD symptoms. The diet of Plp1 transgenic mice was supplemented with curcumin for 10 consecutive weeks followed by phenotypical, histological and immunohistochemical analyses of the central nervous system. Plp1 transgenic and wild-type mice fed with normal chow served as controls. Curcumin improved the motor phenotype performance of Plp1 transgenic mice by 50% toward wild-type level and preserved myelinated axons by 35% when compared to Plp1 transgenic controls. Furthermore, curcumin reduced astrocytosis, microgliosis and lymphocyte infiltration in Plp1 transgenic mice. Curcumin diet did not affect the pathologically increased Plp1 mRNA abundance. However, high glutathione levels indicating an oxidative misbalance in the white matter of Plp1 transgenic mice were restored by curcumin treatment. Curcumin may potentially serve as an antioxidant therapy of PMD caused by PLP...Continue Reading

References

May 20, 1998·The Journal of Comparative Neurology·T J AndersonI R Griffiths
Dec 10, 1999·Annals of the New York Academy of Sciences·T J AndersonI R Griffiths
May 20, 2003·Biological Chemistry·Ralf Dringen, Johannes Hirrlinger
Jan 27, 2005·Investigative Ophthalmology & Visual Science·Pamela Maher, Anne Hanneken
Oct 20, 2006·Cancer Chemotherapy and Pharmacology·Timothy H MarczyloAndreas J Gescher
Sep 29, 2007·Biochemical Pharmacology·Ajay GoelBharat B Aggarwal
May 20, 2008·Expert Reviews in Molecular Medicine·Karen J Woodward
Mar 11, 2009·Frontiers in Bioscience (Landmark Edition)·Vittorio CalabreseAlbena T Dinkova Kostova
Jan 22, 2010·EMBO Molecular Medicine·Julia M EdgarKlaus-Armin Nave
Nov 12, 2010·Nature·Klaus-Armin Nave
Mar 16, 2011·Journal of Anatomy·Fredrik I GruenenfelderJulia M Edgar
Mar 19, 2011·Journal of Natural Products·John CuomoBrian M Dixon
Mar 23, 2012·Molecular Genetics and Metabolism·Li-Hua YuKen Inoue
Jun 19, 2012·Nature Medicine·Gesine SaherKlaus-Armin Nave
Jul 10, 2012·Journal of Neuroinflammation·Philipp AlbrechtAxel Methner
Jan 3, 2013·BioFactors·Adeeb ShehzadYoung Sup Lee
Jan 11, 2013·BioFactors·Adriana MonroySilvestre Alavez
Apr 1, 2014·American Journal of Human Genetics·Thomas PrukopMichael W Sereda
Oct 1, 1999·Annals of the New York Academy of Sciences·T J AndersonI R Griffiths

❮ Previous
Next ❯

Methods Mentioned

BETA
transgenic
PCR
scraping

Software Mentioned

GraphPad
GraphPad Prism

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.