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lüll Mitochondrial ATP synthase subunit c stored in hereditary ceroid-lipofuscinosis contains trimethyl-lysine Katz ML; Gao CL; Tompkins JA; Bronson RT; Chin DTBiochem J 1995[Sep]; 310 ( Pt 3) (Pt 3): 887-92The subunit c protein of mitochondrial ATP synthase accumulates in lysosomal storage bodies of numerous tissues in human subjects with certain forms of ceroid-lipofuscinosis, a degenerative hereditary disease. Subunit c appears to constitute a major fraction of the total storage-body protein. Lysosomal accumulation of subunit c has also been reported in putative animal models (dogs, sheep and mice) for ceroid-lipofuscinosis. In humans with the juvenile form of the disease, hydrolysates of total storage-body protein have been found to contain significant amounts of epsilon-N-trimethyl-lysine (TML). TML is also abundant in storage-body protein hydrolysates from affected dogs and sheep. These findings suggested that one or both of the two lysine residues of subunit c might be methylated in the stored form of the protein. The normal subunit c protein from mitochondria does not appear to be methylated. In a putative canine model for human juvenile ceroid-lipofuscinosis, residue 43 of the storage-body subunit c was previously found to be TML. In the present study, subunit c was isolated from the storage bodies of humans with juvenile ceroid-lipofuscinosis, and from sheep and mice with apparently analogous diseases. In all three species, partial amino acid sequence analysis of the stored subunit c indicated that the protein contained TML at residue 43. These findings strongly suggest that specific methylation of lysine residue 43 of mitochondrial ATP synthase plays a central role in the lysosomal storage of this protein.|Adolescent[MESH]|Adult[MESH]|Amino Acid Sequence[MESH]|Animals[MESH]|Autopsy[MESH]|Brain/*enzymology/pathology[MESH]|Cytoplasmic Granules/enzymology/pathology/ultrastructure[MESH]|Electrophoresis, Polyacrylamide Gel[MESH]|Humans[MESH]|Lysine/*analogs & derivatives/analysis[MESH]|Macromolecular Substances[MESH]|Mice[MESH]|Mitochondria/*enzymology[MESH]|Molecular Sequence Data[MESH]|Neuronal Ceroid-Lipofuscinoses/*enzymology/*genetics/veterinary[MESH]|Proton-Translocating ATPases/*chemistry/genetics/isolation & purification[MESH]|Rodent Diseases[MESH]|Sheep[MESH]|Sheep Diseases[MESH] |