Recombinant protein encompassing a sequence within the center region of human LRP130. The exact sequence is proprietary.
Alternative Synonym:
LSFC, GP130, LRP130, MC4DN5, CLONE-23970
This gene encodes a leucine-rich protein that has multiple pentatricopeptide repeats (PPR). The precise role of this protein is unknown but studies suggest it may play a role in cytoskeletal organization, vesicular transport, or in transcriptional regulation of both nuclear and mitochondrial genes. The protein localizes primarily to mitochondria and is predicted to have an N-terminal mitochondrial targeting sequence. Mutations in this gene are associated with the French-Canadian type of Leigh syndrome.
