Volltext-Downloads (blau) und Frontdoor-Views (grau)

A monoclonal antibody raised against bacterially expressed MPV17 sequences shows peroxisomal, endosomal and lysosomal localisation in U2OS cells

  • Recessive mutations in the MPV17 gene cause mitochondrial DNA depletion syndrome, a fatal infantile genetic liver disease in humans. Loss of function in mice leads to glomerulosclerosis and sensineural deafness accompanied with mitochondrial DNA depletion. Mutations in the yeast homolog Sym1, and in the zebra fish homolog tra cause interesting, but not obviously related phenotypes, although the human gene can complement the yeast Sym1 mutation. The MPV17 protein is a hydrophobic membrane protein of 176 amino acids and unknown function. Initially localised in murine peroxisomes, it was later reported to be a mitochondrial inner membrane protein in humans and in yeast. To resolve this contradiction we tested two new mouse monoclonal antibodies directed against the human MPV17 protein in Western blots and immunohistochemistry on human U2OS cells. One of these monoclonal antibodies showed specific reactivity to a protein of 20 kD absent in MPV17 negative mouse cells. Immunofluorescence studies revealed colocalisation with peroxisomal, endosomal and lysosomal markers, but not with mitochondria. This data reveal a novel connection between a possible peroxisomal/endosomal/lysosomal function and mitochondrial DNA depletion.

Download full text files

Export metadata

Additional Services

Share in Twitter Search Google Scholar Check availability


Show usage statistics
Document Type:Article
Author:Hans Weiher, Haymo Pircher, Pidder Jansen-Dürr, Silke Hegenbarth, Percy Knolle, Silke Grunau, Miia Vapola, J. Kalervo Hiltunen, Ralf M. Zwacka, Elmon Schmelzer, Kerstin Reumann, Hans Will
Parent Title (English):BMC Research Notes
First Page:128
Publisher:BioMed Central
Publishing Institution:Hochschule Bonn-Rhein-Sieg
Date of first publication:2016/02/27
Copyright:© 2016 Weiher et al. This article is distributed under the terms of the Creative Commons Attribution 4.0 International License.
Keyword:Endosomes; Lysosomes; MPV17 monoclonal antibody; Mitochondria; Mitochondrial DNA depletion syndrome; Peroxisomes
Departments, institutes and facilities:Fachbereich Angewandte Naturwissenschaften
Institut für funktionale Gen-Analytik (IFGA)
Dewey Decimal Classification (DDC):5 Naturwissenschaften und Mathematik / 57 Biowissenschaften; Biologie / 570 Biowissenschaften; Biologie
Entry in this database:2016/03/03
Licence (German):License LogoCreative Commons - CC BY - Namensnennung 4.0 International