LEU5/YHR002W Summary Help

Standard Name LEU5 1
Systematic Name YHR002W
Feature Type ORF, Verified
Description Mitochondrial carrier protein; involved in the accumulation of CoA in the mitochondrial matrix; homolog of human Graves disease protein; does not encode an isozyme of Leu4p, as first hypothesized (2 and see Summary Paragraph)
Name Description LEUcine biosynthesis 3
Chromosomal Location
ChrVIII:108812 to 109885 | ORF Map | GBrowse
Gene Ontology Annotations All LEU5 GO evidence and references
  View Computational GO annotations for LEU5
Molecular Function
Manually curated
Biological Process
Manually curated
Cellular Component
Manually curated
Regulators 2 genes
Classical genetics
Large-scale survey
42 total interaction(s) for 42 unique genes/features.
Physical Interactions
  • PCA: 1

Genetic Interactions
  • Negative Genetic: 26
  • Positive Genetic: 15

Expression Summary
Length (a.a.) 357
Molecular Weight (Da) 40,825
Isoelectric Point (pI) 10.41
Phosphorylation PhosphoGRID | PhosphoPep Database
sequence information
ChrVIII:108812 to 109885 | ORF Map | GBrowse
Last Update Coordinates: 2011-02-03 | Sequence: 1996-07-31
Subfeature details
Most Recent Updates
Coordinates Sequence
CDS 1..1074 108812..109885 2011-02-03 1996-07-31
Retrieve sequences
Analyze Sequence
S288C only
S288C vs. other species
S288C vs. other strains
External Links All Associated Seq | Entrez Gene | Entrez RefSeq Protein | MIPS | Search all NCBI (Entrez) | UniProtKB
Primary SGDIDS000001044

LEU5 encodes a mitochondrial inner membrane protein that is involved in the transport of coenzyme A (CoA) to the mitochondrial matrix (2). It was originally thought that LEU5 encoded the minor isozyme of isopropylmalate synthase (1), but further analysis showed that Leu5p was not directly involved in leucine biosynthesis and was instead similar to mitochondrial carrier proteins (3, 4, and 2). Leu5p has sequence features characteristic of the mitochondrial carrier family: it lacks a typical N-terminal targeting sequence for mitochondrial matrix localization, and exhibits a tripartite structure with three repetitive domains, each comprised of two transmembrane regions and a carrier signature motif (4 and 2 and references therein).

Mutants lacking Leu5p activity grow poorly on non-fermentable carbon sources, such as glycerol, and exhibit a 15-fold decrease in mitochondrial CoA levels compared to wild-type cells (3, 2). A leu4 leu5 double mutant is synthetically auxotrophic for leucine (1). Leu5p has sequence similarity to S. pombe, bovine, and human proteins; expression of the human homolog SLC25A16 (OMIM), an autoantigen linked to Graves disease (OMIM), is able to rescue leu5 null cells (2 and references therein).

Last updated: 2006-08-10 Contact SGD

References cited on this page View Complete Literature Guide for LEU5
1) Baichwal VR, et al.  (1983) Leucine biosynthesis in yeast : Identification of two genes (LEU4, LEU5) that affect alpha-Isopropylmalate synthase activity and evidence that LEU1 and LEU2 gene expression is controlled by alpha-Isopropylmalate and the product of a regulatory gene. Curr Genet 7(5):369-77
2) Prohl C, et al.  (2001) The yeast mitochondrial carrier Leu5p and its human homologue Graves' disease protein are required for accumulation of coenzyme A in the matrix. Mol Cell Biol 21(4):1089-97
3) Drain P and Schimmel P  (1986) Yeast LEU5 is a PET-like gene that is not essential for leucine biosynthesis. Mol Gen Genet 204(3):397-403
4) El Moualij B, et al.  (1997) Phylogenetic classification of the mitochondrial carrier family of Saccharomyces cerevisiae. Yeast 13(6):573-81