The mitochondrial TIM22 preprotein translocase is highly conserved throughout the eukaryotic kingdom

被引：68

作者：

Bauer, MF

Rothbauer, U

Mühlenbein, N

Smith, RJH

Gerbitz, KD

Neupert, W

Brunner, M

Hofmann, S

机构：

[1] Akad Krankenhaus Munchen Schwabing, Inst Klin Chem Mol Diagnost & Mitochondriale Gene, D-80804 Munich, Germany

[2] Akad Krankenhaus Munchen Schwabing, Inst Diabet Forsch, D-80804 Munich, Germany

[3] Univ Munich, Inst Physiol Chem, D-80336 Munich, Germany

[4] Univ Iowa Hosp & Clin, Dept Otolaryngol, Iowa City, IA 52242 USA

来源：

FEBS LETTERS | 1999年 / 464卷 / 1-2期

关键词：

mitochondrial inner membrane translocase; TIM22; deafness/dystonia peptide 1/Tim10 protein family; Mohr-Tranebjaerg syndrome;

D O I：

10.1016/S0014-5793(99)01665-8

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

The Mohr-Tranebjaerg syndrome (MTS), a neurodegenerative syndrome characterized by progressive sensorineural hearing loss, dystonia, mental retardation and blindness, is a mitochondrial disease caused by mutations in the deafness/dystonia peptide 1 (DDP1) gene, DDP1 shows similarity to the yeast proteins Tim9, Tim10 and Tim12, components of the mitochondrial import machinery for carrier proteins, Here, we show that DDP1 belongs to a large family of evolutionarily conserved proteins. We report the identification, chromosomal localization and expressional analysis of six human family members which represent further candidate genes for neurodegenerative diseases. (C) 1999 Federation of European Biochemical Societies.

引用

页码：41 / 47

页数：7

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