Distinct effects of tafazzin deletion in differentiated and undifferentiated mitochondria

被引：61

作者：

Acehan, Devrim ^{[2
,3
]}

Khuchua, Zaza ^{[5
]}

Houtkooper, Riekelt H. ^{[6
]}

Malhotra, Ashim

Kaufman, Johanna

Vaz, Frederic M. ^{[6
]}

Ren, Mindong ^{[3
]}

Rockman, Howard A. ^{[7
]}

Stokes, David L. ^{[2
,3
,4
]}

Schlame, Michael ^{[1
,3
]}

机构：

[1] NYU, Sch Med, Dept Anesthesiol, Langone Med Ctr, New York, NY 10016 USA

[2] NYU, Langone Med Ctr, Skirball Inst, New York, NY 10016 USA

[3] NYU, Langone Med Ctr, Dept Cell Biol, New York, NY 10016 USA

[4] New York Struct Biol Ctr, New York, NY 10027 USA

[5] Cincinnati Childrens Hosp, Med Ctr, Dept Pediat, Cincinnati, OH 45229 USA

[6] Univ Amsterdam, Dept Clin Chem, NL-1100 DE Amsterdam, Netherlands

[7] Duke Univ, Dept Med, Durham, NC 27110 USA

来源：

MITOCHONDRION | 2009年 / 9卷 / 02期

基金：

美国国家卫生研究院; 美国国家科学基金会;

关键词：

Tafazzin; Cardiolipin; Mitochondria; Barth syndrome; Electron microscopy; Electron tomography; LINKED CARDIOSKELETAL MYOPATHY; NEUTROPENIA BARTH-SYNDROME; LYMPHOBLAST MITOCHONDRIA; DILATED CARDIOMYOPATHY; SKELETAL-MUSCLE; MIM; 302060; CARDIOLIPIN; DROSOPHILA; MODEL; APOPTOSIS;

D O I：

10.1016/j.mito.2008.12.001

中图分类号：

Q2 [细胞生物学];

学科分类号：

071009 ; 090102 ;

摘要：

Tafazzin is a conserved mitochondrial protein that is required to maintain normal content and composition of cardiolipin. We used electron tomography to investigate the effect of tafazzin deletion on mitochondrial structure and found that cellular differentiation plays a crucial role in the manifestation of abnormalities. This conclusion was reached by comparing differentiated cardiomyocytes with embryonic stem cells from mouse and by comparing different tissues from Drosophila melanogaster. The data suggest that tafazzin deficiency affects cardiolipin in all mitochondria, but significant alterations of the ultrastructure, such as remodeling and aggregation of inner membranes, will only occur after specific differentiation. (C) 2008 Elsevier B.V. and Mitochondria Research Society. All rights reserved.

引用

页码：86 / 95

页数：10

共 40 条

[1] Comparison of lymphoblast mitochondria from normal subjects and patients with Barth syndrome using electron microscopic tomography [J].

Acehan, Devrim ;

Xu, Yang ;

Stokes, David L. ;

Schlame, Michael .

LABORATORY INVESTIGATION, 2007, 87 (01) :40-48

[2] AN X-LINKED MITOCHONDRIAL DISEASE AFFECTING CARDIAC-MUSCLE, SKELETAL-MUSCLE AND NEUTROPHIL LEUKOCYTES [J].

BARTH, PG ;

SCHOLTE, HR ;

BERDEN, JA ;

VANDERKLEIVANMOORSEL, JM ;

LUYTHOUWEN, IEM ;

VANTVEERKORTHOF, ET ;

VANDERHARTEN, JJ ;

SOBOTKAPLOJHAR, MA .

JOURNAL OF THE NEUROLOGICAL SCIENCES, 1983, 62 (1-3) :327-355

[3] X-linked cardioskeletal myopathy and neutropenia (Barth syndrome) (MIM 302060) [J].

Barth, PG ;

Wanders, RJA ;

Vreken, P ;

Janssen, EAM ;

Lam, J ;

Baas, F .

JOURNAL OF INHERITED METABOLIC DISEASE, 1999, 22 (04) :555-567

[4] BEHAVIORAL MUTANTS OF DROSOPHILA ISOLATED BY COUNTERCURRENT DISTRIBUTION [J].

BENZER, S .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1967, 58 (03) :1112-&

[5] Taz1, an outer mitochondrial membrane protein, affects stability and assembly of inner membrane protein complexes: Implications for Barth syndrome [J].

Brandner, K ;

Mick, DU ;

Frazier, AE ;

Taylor, RD ;

Meisinger, C ;

Rehling, P .

MOLECULAR BIOLOGY OF THE CELL, 2005, 16 (11) :5202-5214

[6] BARTH SYNDROME - CLINICAL OBSERVATIONS AND GENETIC-LINKAGE STUDIES [J].

CHRISTODOULOU, J ;

MCINNES, RR ;

JAY, V ;

WILSON, G ;

BECKER, LE ;

LEHOTAY, DC ;

PLATT, BA ;

BRIDGE, PJ ;

ROBINSON, BH ;

CLARKE, JTR .

AMERICAN JOURNAL OF MEDICAL GENETICS, 1994, 50 (03) :255-264

[7] The internal structure of mitochondria [J].

Frey, TG ;

Mannella, CA .

TRENDS IN BIOCHEMICAL SCIENCES, 2000, 25 (07) :319-324

[8]

Greenawalt J W, 1974, Methods Enzymol, V31, P310

[9] Aberrant cardiolipin metabolism in the yeast taz1 mutant:: a model for Barth syndrome [J].

Gu, ZM ;

Valianpour, F ;

Chen, SL ;

Vaz, FM ;

Hakkaart, GA ;

Wanders, RJA ;

Greenberg, ML .

MOLECULAR MICROBIOLOGY, 2004, 51 (01) :149-158

[10] Identification and characterization of human cardiolipin synthase [J].

Houtkooper, Riekelt H. ;

Akbari, Hana ;

van Lenthe, Henk ;

Kulik, Willem ;

Wanders, Ronald J. A. ;

Frentzen, Margrit ;

Vaz, Frederic M. .

FEBS LETTERS, 2006, 580 (13) :3059-3064

← 1 2 3 4 →