Decreased turnover of the CNS myelin protein Opalin in a mouse model of hereditary spastic paraplegia 35.

Human Molecular Genetics
Robert HardtMatthias Eckhardt

Abstract

Spastic paraplegia 35 (SPG35) (OMIM: 612319) or fatty acid hydroxylase-associated neurodegeneration (FAHN) is caused by deficiency of fatty acid 2-hydroxylase (FA2H). This enzyme synthesizes sphingolipids containing 2-hydroxylated fatty acids, which are particularly abundant in myelin. Fa2h-deficient (Fa2h-/-) mice develop symptoms reminiscent of the human disease and therefore serve as animal model of SPG35. In order to understand further the pathogenesis of SPG35, we compared the proteome of purified CNS myelin isolated from wild type and Fa2h-/- mice at different time points of disease progression using tandem mass tag labeling. Data analysis with a focus on myelin membrane proteins revealed a significant increase of the oligodendrocytic myelin paranodal and inner loop protein (Opalin) in Fa2h-/- mice, whereas the concentration of other major myelin proteins was not significantly changed. Western blot analysis revealed an almost 6-fold increase of Opalin in myelin of Fa2h-/- mice aged 21-23 months. A concurrent unaltered Opalin gene expression suggested a decreased turnover of the Opalin protein in Fa2h-/- mice. Supporting this hypothesis, Opalin protein half-life was reduced significantly when expressed in CHO cells synthes...Continue Reading

References

Apr 1, 1995·Neuropathology and Applied Neurobiology·I R GriffithsP Montague
Jun 18, 2002·Journal of Neurochemistry·Christopher M TaylorSteven E Pfeiffer
Aug 15, 2002·The Biochemical Journal·Matthias EckhardtVolkmar Gieselmann
Sep 1, 2004·The Journal of Biological Chemistry·Nathan L AldersonHiroko Hama
Mar 1, 2005·The European Journal of Neuroscience·Afshin YaghootfamMatthias Eckhardt
Apr 13, 2005·The Biochemical Journal·Yukiko MizutaniYasuyuki Igarashi
May 11, 2005·The Journal of Cell Biology·Delphine DelacourGuillemette Huet
Sep 26, 2006·Journal of Lipid Research·Nathan L AldersonHiroko Hama
Jul 31, 2007·Biochemistry·Afshin YaghootfamMatthias Eckhardt
Aug 24, 2007·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Matthias EckhardtVolkmar Gieselmann
Sep 29, 2007·Journal of Lipid Research·Eduardo N MaldonadoHiroko Hama
Jan 30, 2008·Current Protocols in Cell Biology·Jorge N Larocca, Williams T Norton
Feb 28, 2008·Journal of Cell Science·Christine WintersteinEva-Maria Krämer-Albers
May 10, 2008·Molecular Neurobiology·Matthias Eckhardt
May 21, 2008·The Journal of Biological Chemistry·Fumio YoshikawaTeiichi Furuichi
Sep 26, 2008·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Inge ZöllerMatthias Eckhardt
Dec 6, 2008·Brain : a Journal of Neurology·Jennifer L Orthmann-MurphyDavide Pareyson
May 20, 2009·Molecular Neurobiology·Olaf JahnHauke B Werner
Oct 29, 2009·Journal of Neurochemistry·Rebekka van ZylMatthias Eckhardt
Dec 23, 2009·Biochimica Et Biophysica Acta·Hiroko Hama
Jan 28, 2010·Human Mutation·Katherine J DickAndrew H Crosby
Sep 21, 2010·Annals of Neurology·Michael C KruerSusan J Hayflick
Apr 15, 2011·Glia·Kathleen A PotterHiroko Hama
Jun 2, 2011·The Journal of Biological Chemistry·Helena MaierMatthias Eckhardt
Mar 24, 2012·Cellular and Molecular Life Sciences : CMLS·Patricia de Monasterio-SchraderHauke B Werner
Apr 4, 2012·Current Protocols in Protein Science·Sean R Gallagher
Dec 4, 2012·Nucleic Acids Research·Juan Antonio VizcaínoHenning Hermjakob
Oct 22, 2013·Advances in Biological Regulation·Venkatesh Kota, Hiroko Hama

❮ 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.