Protein Name leucine rich pentatricopeptide repeat containing
Organism Homo sapiens
Gene ID 10128
Gene Symbol

LRPPRC

UniProt P42704 (LPPRC_HUMAN), E5KNY5 (E5KNY5_HUMAN)
Relationships Total Number of functionally related compound(s) : 34
Total Number of Articles : 33
Description

leucine rich pentatricopeptide repeat containing

Gene Summary

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. [provided by RefSeq, Mar 2012]

synonyms
  • leucine-rich PPR motif-containing protein, mitochondrial
  • 130 kDa leucine-rich protein
  • LRP 130
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Properties