Leucine Rich PPR Motif Containing Protein (LRPPRC)

LRP130; LSFC; GP130; 130 kDa leucine-rich protein; Leigh Syndrome,French-Canadian Type(Cytochrome Oxidase Deficiency)

Leucine Rich PPR Motif Containing Protein (LRPPRC)
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)

Organism species: Mus musculus (Mouse)

Organism species: Rattus norvegicus (Rat)