title: Heterogeneity of Matrin 3 in the developing and aging murine central nervous system.
abstract: Mutations in the MATR3 gene encoding the nucleotide binding protein Matrin 3 have recently been identified as causing a subset of familial amyotrophic lateral sclerosis (fALS) and more rarely causing distal myopathy. Translating the identification of MATR3 mutations into an understanding of disease pathogenesis and the creation of mouse models requires a complete understanding of normal Matrin 3 levels and distribution in vivo. Consequently, we examined the levels of murine Matrin 3 in body tissues and regions of the central nervous system (CNS). We observed a significant degree of variability in Matrin 3 protein levels among different tissues of adult animals, with the highest levels found in reproductive organs and the lowest in muscle. Within the adult CNS, Matrin 3 levels were lowest in spinal cord. Further, we found that Matrin 3 declines significantly in CNS through early development and young adulthood before stabilizing. As previously reported, antibodies to Matrin 3 primarily stain nuclei, but the intensity of staining was not uniform in all nuclei. The low levels of Matrin 3 in spinal cord and muscle could mean that that these tissues are particularly vulnerable to alterations in Matrin 3 function. Our study is the first to characterize endogenous Matrin 3 in rodents across the lifespan, providing the groundwork for deciphering disease mechanisms and developing mouse models of MATR3-linked ALS. J. Comp. Neurol. 524:2740-2752, 2016. © 2016 Wiley Periodicals, Inc.
authors.affiliation: Center for Translational Research in Neurodegenerative Disease, University of Florida, Gainesville, Florida, USA.|Department of Neuroscience, University of Florida, Gainesville, Florida, USA.
authors.forename: Sruti
authors.initials: S
authors.lastname: Rayaprolu
authors.affiliation: Center for Translational Research in Neurodegenerative Disease, University of Florida, Gainesville, Florida, USA.|Department of Neuroscience, University of Florida, Gainesville, Florida, USA.
authors.forename: Simon
authors.initials: S
authors.lastname: D'Alton
authors.affiliation: Center for Translational Research in Neurodegenerative Disease, University of Florida, Gainesville, Florida, USA.|Department of Neuroscience, University of Florida, Gainesville, Florida, USA.
authors.forename: Keith
authors.initials: K
authors.lastname: Crosby
authors.affiliation: Center for Translational Research in Neurodegenerative Disease, University of Florida, Gainesville, Florida, USA.|Department of Neuroscience, University of Florida, Gainesville, Florida, USA.
authors.forename: Christina
authors.initials: C
authors.lastname: Moloney
authors.affiliation: Center for Translational Research in Neurodegenerative Disease, University of Florida, Gainesville, Florida, USA.|Department of Neuroscience, University of Florida, Gainesville, Florida, USA.
authors.forename: John
authors.initials: J
authors.lastname: Howard
authors.affiliation: Center for Translational Research in Neurodegenerative Disease, University of Florida, Gainesville, Florida, USA.|Department of Neuroscience, University of Florida, Gainesville, Florida, USA.
authors.forename: Colin
authors.initials: C
authors.lastname: Duffy
authors.affiliation: Center for Translational Research in Neurodegenerative Disease, University of Florida, Gainesville, Florida, USA.|Department of Neuroscience, University of Florida, Gainesville, Florida, USA.
authors.forename: Mariela
authors.initials: M
authors.lastname: Cabrera
authors.affiliation: Center for Translational Research in Neurodegenerative Disease, University of Florida, Gainesville, Florida, USA.|Department of Neuroscience, University of Florida, Gainesville, Florida, USA.
authors.forename: Zoe
authors.initials: Z
authors.lastname: Siemienski
authors.affiliation: Center for Translational Research in Neurodegenerative Disease, University of Florida, Gainesville, Florida, USA.|Department of Neuroscience, University of Florida, Gainesville, Florida, USA.
authors.forename: Abigail R
authors.initials: AR
authors.lastname: Hernandez
authors.affiliation: Center for Translational Research in Neurodegenerative Disease, University of Florida, Gainesville, Florida, USA.|Department of Neuroscience, University of Florida, Gainesville, Florida, USA.
authors.forename: Carolina
authors.initials: C
authors.lastname: Gallego-Iradi
authors.affiliation: Center for Translational Research in Neurodegenerative Disease, University of Florida, Gainesville, Florida, USA.|Department of Neuroscience, University of Florida, Gainesville, Florida, USA.|McKnight Brain Institute, Department of Neuroscience, University of Florida, Gainesville, Florida, USA.
authors.forename: David R
authors.initials: DR
authors.lastname: Borchelt
authors.affiliation: Center for Translational Research in Neurodegenerative Disease, University of Florida, Gainesville, Florida, USA.|Department of Neuroscience, University of Florida, Gainesville, Florida, USA.|McKnight Brain Institute, Department of Neuroscience, University of Florida, Gainesville, Florida, USA.
authors.forename: Jada
authors.initials: J
authors.lastname: Lewis
mesh_terms: D000375:Aging / Q000378:metabolism*; D000818:Animals; D001921:Brain / Q000254:growth & development* / Q000378:metabolism*; D002490:Central Nervous System / Q000254:growth & development / Q000378:metabolism; D005260:Female; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D034521:Nuclear Matrix-Associated Proteins / Q000096:biosynthesis*; D016601:RNA-Binding Proteins / Q000096:biosynthesis*; D013116:Spinal Cord / Q000254:growth & development* / Q000378:metabolism*
chemical_list: D034521:Nuclear Matrix-Associated Proteins; D016601:RNA-Binding Proteins; C573692:matrin-3 protein, mouse
keywords: Matrin 3; RRID: 151542; RRID: AB11128483; RRID: AB525453; SCR_013724; aging; amyotrophic lateral sclerosis; development; neurodegeneration