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Article

Pharmacologic rescue of an enzyme-trafficking defect in primary hyperoxaluria 1.

Proceedings of the National Academy of Sciences of the United States of America - 7 Oct 2014

Miyata Non, Steffen Janos, Johnson Meghan E, Fargue Sonia, Danpure Christopher J, Koehler Carla M

Abstract excerpt

Primary hyperoxaluria 1 (PH1; Online Mendelian Inheritance in Man no. 259900), a typically lethal biochemical disorder, may be caused by the AGT(P11LG170R) allele in which the alanine:glyoxylate aminotransferase (AGT) enzyme is mistargeted from peroxisomes to mitochondria. AGT contains a C-terminal peroxisomal targeting sequence, but mutations generate an N-terminal mitochondrial targeting sequence that directs...

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Identifiers

PMID: 25237136
DOI: 10.1073/pnas.1408401111
PMCID: PMC4210028

Genes

AGT

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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