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Depending on the particular species, AGT can be found either in peroxisomes or mitochondria, or in both peroxisomes and mitochondria. This variable localization depends on the differential expression of N\u2010terminal mitochondrial and C\u2010terminal peroxisomal targeting sequences by the use of alternative transcription and translation initiation sites. AGT is peroxisomal in most humans, but it is mistargeted to the mitochondria in a subset of patients suffering from the rare herediatry disease primary hyperoxaluria type 1. Mistargeting is due to the unlikely combination of a normally occurring polymorphism that generates a functionally weak mitochondrial targeting sequence and a disease\u2010specific mutation which, in combination with the polymorphism, inhibits AGT dimerization. The mechanisms by which AGT can be targeted differentially to peroxisomes and\/or mitochondria highlight the different molecular requirements for protein import into these two organelles.<\/jats:p>","DOI":"10.1002\/bies.950190409","type":"journal-article","created":{"date-parts":[[2005,2,25]],"date-time":"2005-02-25T07:29:02Z","timestamp":1109316542000},"page":"317-326","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":61,"title":["Variable peroxisomal and mitochondrial targeting of alanine: Glyoxylate aminotransferase in mammalian evolution and disease"],"prefix":"10.1002","volume":"19","author":[{"given":"Christopher J.","family":"Danpure","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2005,2,5]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1242\/jcs.97.4.669"},{"key":"e_1_2_1_3_2","first-page":"295","article-title":"Evolution of alanine:glyoxylate aminotransferase 1 peroxisomal and mitochondrial targeting. 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