title: Human pentatricopeptide proteins: only a few and what do they do?
abstract: Pentatricopeptide repeat (PPR) proteins constitute a large family of RNA-binding proteins that contain a canonical 35 residue repeat motif. Originally identified in Arabidopsis thaliana, family members are found in protists, fungi, and metazoan but are by far most abundant in plant organelles. Seven examples have been identified in human mitochondria and roles have been tentatively ascribed to each. In this review, we briefly outline each of these PPR proteins and discuss the role each is believed to play in facilitating mitochondrial gene expression.
authors: The Wellcome Trust Centre for Mitochondrial Research; Institute for Cell and Molecular Biosciences; Newcastle University; The Medical School; Framlington Place; Newcastle upon Tyne, UK.
authors: Robert N
authors: RN
authors: Lightowlers
authors: The Wellcome Trust Centre for Mitochondrial Research; Institute for Ageing and Health; Newcastle University; The Medical School; Framlington Place; Newcastle upon Tyne, UK.
authors: Zofia M A
authors: ZM
authors: Chrzanowska-Lightowlers
mesh_terms: D000595:Amino Acid Sequence; D029681:Arabidopsis Proteins / Q000235:genetics / Q000378:metabolism; D017124:Conserved Sequence; D012321:DNA-Directed RNA Polymerases / Q000235:genetics / Q000378:metabolism; D006801:Humans; D008928:Mitochondria / Q000235:genetics* / Q000378:metabolism; D024101:Mitochondrial Proteins / Q000235:genetics / Q000378:metabolism; D009363:Neoplasm Proteins / Q000737:chemistry / Q000235:genetics / Q000378:metabolism; D012323:RNA Processing, Post-Transcriptional; D016601:RNA-Binding Proteins / Q000235:genetics / Q000378:metabolism*; D043262:Ribonuclease P / Q000235:genetics / Q000378:metabolism; D012269:Ribosomal Proteins / Q000235:genetics / Q000378:metabolism
chemical_list: D029681:Arabidopsis Proteins; C087940:LRPPRC protein, human; C577036:MRPS27 protein, human; D024101:Mitochondrial Proteins; D009363:Neoplasm Proteins; C544933:PTCD1 protein, human; C000597731:PTCD2 protein, human; C000597732:PTCD3 protein, human; D016601:RNA-Binding Proteins; D012269:Ribosomal Proteins; D012321:DNA-Directed RNA Polymerases; C519567:POLRMT protein, human; C545252:PRORP protein, human; D043262:Ribonuclease P
keywords: Pentatricopeptide; RNA binding; human; mitochondria; post-transcriptional gene expression; translation
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