Leucine Rich PPR Motif Containing Protein (LRPPRC)
LRP130; LSFC; GP130; 130 kDa leucine-rich protein; Leigh Syndrome,French-Canadian Type(Cytochrome Oxidase Deficiency)
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LRPPRC contains 167 leucine residues that are dispersed every 15 to 19 amino acids throughout the protein, accounting for about 13% of the sequence. The N-terminal half of LRPPRC is cysteine rich.
LRPPRC has 4 potential N-linked glycosylation sites, a sequence that shares homology with the ATP-binding site of ATP-dependent kinases, and a protein kinase C phosphorylation site. It has no secretory signal sequence or hydrophobic domains. Northern blot analysis of HepG2 cells revealed expression of a major 4.8-kb transcript and minor transcripts of 5.2 and 7.0 kb. Western blot analysis of transiently transfected COS-1 cells revealed a doublet with an apparent molecular mass of about 130 kD.
Organism species: Homo sapiens (Human)
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Organism species: Mus musculus (Mouse)
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Organism species: Rattus norvegicus (Rat)
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