Submitochondrial distribution and delayed proteolysis of subunit c of the H+-transporting ATP-synthase in ovine ceroid-lipofuscinosis.

@article{Hughes2001SubmitochondrialDA,
  title={Submitochondrial distribution and delayed proteolysis of subunit c of the H+-transporting ATP-synthase in ovine ceroid-lipofuscinosis.},
  author={Stephanie M. Hughes and P Moroni-Rawson and Robert D. Jolly and Thomas William Jordan},
  journal={Electrophoresis},
  year={2001},
  volume={22 9},
  pages={1785-94}
}
The neuronal ceroid-lipofuscinose (NCL) are recessively inherited lysosomal storage diseases in children and animals. The major stored protein in many of these diseases is subunit c of the mitochondrial inner membrane H+-transporting ATP-synthase. Previous studies of naturally occurring ovine ceroid-lipofuscinosis (OCL) in South Hampshire sheep showed that the genes and transcripts for subunit c were normal and inferred that this protein was expressed normally in mitochondria prior to storage… CONTINUE READING

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