UCL logo

UCL Discovery

UCL home » Library Services » Electronic resources » UCL Discovery

Variable peroxisomal and mitochondrial targeting of alanine: Glyoxylate aminotransferase in mammalian evolution and disease

Danpure, CJ; (1997) Variable peroxisomal and mitochondrial targeting of alanine: Glyoxylate aminotransferase in mammalian evolution and disease. BIOESSAYS , 19 (4) 317 - 326.

Full text not available from this repository.

Abstract

Under the putative influence of dietary selection pressure, the subcellular distribution of alanine:glyoxylate aminotransferase 1 (AGT) has changed on many occasions during the evolution of mammals. 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-terminal mitochondrial and C-terminal 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 hereditary 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-specific 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.

Type:Article
Title:Variable peroxisomal and mitochondrial targeting of alanine: Glyoxylate aminotransferase in mammalian evolution and disease
Keywords:PRIMARY HYPEROXALURIA TYPE-1, HEAT-SHOCK PROTEIN, HEPATIC ALANINE, RAT-LIVER, PYRUVATE AMINOTRANSFERASE, ZELLWEGER SYNDROME, SUBCELLULAR-DISTRIBUTION, 3-KETOACYL-COA THIOLASE, MOLECULAR CHAPERONES, STRUCTURAL FEATURES
UCL classification:UCL > School of Life and Medical Sciences > Faculty of Life Sciences > Biosciences (Division of)

Archive Staff Only: edit this record