title: The long, but not the short, presequence of human coproporphyrinogen oxidase is essential for its import and sorting to mitochondria.
abstract: Nucleotide sequence analyses of human coproporphyrinogen oxidase (CPO) have shown two possible translation initiation eodons (TICs) in the region upstream from the region coding the mature polypeptide. These TICs lead to the precursor polypeptides of CPO (long and short pCPOs), which have 110 and 10 amino acids (long and short) presequences, respectively. To determine which TIC is the real one, import reactions to mitochondria of the two pCPOs synthesized using in vitro transcription-translation system were analyzed. The results showed that the long pCPO was imported into mitochondria and sorted properly to the intermembrane space, whereas the short pCPO was not. We also found several miss-readings in the reported nucleotide sequence of mouse CPO gene. This correction revealed that mouse pCPO has also a longer presequence, which are very similar to the long presequence of human. These results indicate that the long, but not short, presequence is the real one.
authors.affiliation: The Third Department of Internal Medicine, Yamagata University School of Medicine, Yamagata 990-9585, Japan.
authors.forename: Shinji
authors.initials: S
authors.lastname: Susa
authors.forename: Makoto
authors.initials: M
authors.lastname: Daimon
authors.forename: Hideyu
authors.initials: H
authors.lastname: Ono
authors.forename: Song
authors.initials: S
authors.lastname: Li
authors.forename: Tadashi
authors.initials: T
authors.lastname: Yoshida
authors.forename: Takeo
authors.initials: T
authors.lastname: Kato
mesh_terms: D020121:5' Untranslated Regions / Q000235:genetics; D000595:Amino Acid Sequence; D000818:Animals; D001483:Base Sequence; D003304:Coproporphyrinogen Oxidase / Q000235:genetics* / Q000378:metabolism*; D017931:DNA Primers; D018076:DNA, Complementary / Q000096:biosynthesis / Q000235:genetics; D006801:Humans; D006868:Hydrolysis; D051379:Mice; D008928:Mitochondria / Q000201:enzymology*; D008969:Molecular Sequence Data; D020133:Reverse Transcriptase Polymerase Chain Reaction; D013045:Species Specificity; D014357:Trypsin / Q000737:chemistry
chemical_list: D020121:5' Untranslated Regions; D017931:DNA Primers; D018076:DNA, Complementary; D003304:Coproporphyrinogen Oxidase; D014357:Trypsin