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

Weiher, Hans and Pircher, Haymo and Jansen-Dürr, Pidder and Hegenbarth, Silke and Knolle, Percy and Grunau, Silke and Vapola, Miia and Hiltunen, J Kalervo and Zwacka, Ralf M and Schmelzer, Elmon and Reumann, Kerstin and Will, Hans (2016) 'A monoclonal antibody raised against bacterially expressed MPV17 sequences shows peroxisomal, endosomal and lysosomal localisation in U2OS cells.' BMC Research Notes, 9 (1). 128-. ISSN 1756-0500

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Abstract

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.

Item Type: Article
Uncontrolled Keywords: Cell Line, Tumor; Peroxisomes; Endosomes; Lysosomes; Mitochondria; Fibroblasts; Osteoblasts; Animals; Humans; Mice; Membrane Proteins; Mitochondrial Proteins; Recombinant Proteins; DNA, Mitochondrial; Antibodies, Monoclonal; Fluorescent Antibody Technique; Gene Expression; Mutation
Subjects: Q Science > QP Physiology
Divisions: Faculty of Science and Health
Faculty of Science and Health > Life Sciences, School of
SWORD Depositor: Elements
Depositing User: Elements
Date Deposited: 16 May 2016 08:18
Last Modified: 15 Jan 2022 00:34
URI: http://repository.essex.ac.uk/id/eprint/16678

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