Characterization of a novel human putative mitochondrial transporter homologous to the yeast mitochondrial RNA splicing proteins 3 and 4

F Y Li; K Nikali; J Gregan; I Leibiger; B Leibiger; R Schweyen; C Larsson; A Suomalainen

doi:10.1016/s0014-5793(01)02319-5

Characterization of a novel human putative mitochondrial transporter homologous to the yeast mitochondrial RNA splicing proteins 3 and 4

FEBS Lett. 2001 Apr 6;494(1-2):79-84. doi: 10.1016/s0014-5793(01)02319-5.

Authors

F Y Li¹, K Nikali, J Gregan, I Leibiger, B Leibiger, R Schweyen, C Larsson, A Suomalainen

Affiliation

¹ Department of Molecular Medicine, CMM, Karolinska Hospital, Stockholm, Sweden. fangyuan.li@cmm.ki.se

PMID: 11297739
DOI: 10.1016/s0014-5793(01)02319-5

Abstract

We report here a novel human gene, hMRS3/4, encoding a putative mitochondrial transporter structurally and functionally homologous to the yeast mitochondrial RNA splicing proteins 3 and 4. These proteins belong to the family of mitochondrial carrier proteins (MCF) and are likely to function as solute carriers. hMRS3/4 spans approximately 10 kb of genomic DNA on chromosome 10q24 and consists of four exons that encode a 364-aa protein with six transmembrane domains. A putative splice variant, encoding a 177-aa protein with three transmembrane domains, was also identified. hMRS3/4 has a well-conserved signature sequence of MCF and is targeted into the mitochondria. When expressed in yeast, hMRS3/4 efficiently restores the mitochondrial functions in mrs3(o)mrs4(o) knock-out mutants. Ubiquitous expression in human tissues and a well-conserved structure and function suggest an important role for hMRS3/4 in human cells.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Alternative Splicing*
Amino Acid Sequence
Base Sequence
Carrier Proteins / genetics*
Carrier Proteins / metabolism
Cation Transport Proteins*
Chromosome Mapping
Chromosomes, Human, Pair 10*
DNA, Complementary
Fungal Proteins / genetics
Gene Expression Profiling
Humans
Membrane Transport Proteins*
Mitochondria / metabolism*
Mitochondrial Proteins
Molecular Sequence Data
Mutagenesis
Ophthalmoplegia, Chronic Progressive External / genetics
RNA Splicing
Repressor Proteins*
Saccharomyces cerevisiae / genetics
Saccharomyces cerevisiae Proteins*
Spinocerebellar Ataxias / genetics
Tissue Distribution
Transfection

Substances

Carrier Proteins
Cation Transport Proteins
DNA, Complementary
Fungal Proteins
MRS3 protein, S cerevisiae
MRS4 protein, S cerevisiae
Membrane Transport Proteins
Mitochondrial Proteins
Repressor Proteins
SLC25A28 protein, human
Saccharomyces cerevisiae Proteins

Associated data

GENBANK/AF327402
GENBANK/AF327403
GENBANK/AJ303077
GENBANK/AJ303078