J. Lipid Res.
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
 QUICK SEARCH:   [advanced]


     


This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Schlame, M.
Right arrow Articles by Blanck, T. J. J.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Schlame, M.
Right arrow Articles by Blanck, T. J. J.
Social Bookmarking
 Add to CiteULike   Add to Complore   Add to Connotea   Add to Del.icio.us   Add to Digg   Add to Reddit   Add to Technorati  
What's this?
Journal of Lipid Research, Vol. 40, 1585-1592, September 1999
Copyright © 1999 by Lipid Research, Inc.


Original Article

Microanalysis of cardiolipin in small biopsies including skeletal muscle from patients with mitochondrial disease

Michael Schlamea, Sara Shansked, Steven Dotyb, Thomas Königa, Thomas Sculcoc, Salvatore DiMaurod, and Thomas J. J. Blancka
a Departments of Anesthesiology, Hospital for Special Surgery, Cornell University Medical College, 535 E. 70th Street, New York, NY
b Pathology, Hospital for Special Surgery, Cornell University Medical College, 535 E. 70th Street, New York, NY
c Orthopedic Surgery, Hospital for Special Surgery, Cornell University Medical College, 535 E. 70th Street, New York, NY
d Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, NY

Correspondence to: Thomas J. J. Blanck

Cardiolipin is a specific mitochondrial phospholipid that is present in mammalian tissues in low concentration. To measure cardiolipin in small biopsies from patients with mitochondrial disease, we developed a new technique that can detect subnanomolar levels of well-resolved molecular species, the most abundant of which are tetralinoleoyl-cardiolipin (L4) and trilinoleoyl-oleoyl-cardiolipin (L3O). To this end, a fluorescence-labeled derivative of cardiolipin (2-[naphthyl-1'-acetyl]-cardiolipin dimethyl ester) was formed and analyzed by high performance liquid chromatography. Cardiolipin was measured in skeletal muscle biopsies from 8 patients with mitochondrial disease and in 17 control subjects. In 5 patients with mitochondrial disease, cardiolipin content was higher than normal (2.4;–7.0 vs. 0.4;–2.2 nmol/mg protein). In 3 patients with mitochondrial disease, the L4/L3O ratio was lower than normal (2;–4 vs. 4;–6). Cardiolipin was also measured in various rat and dog muscle tissues. The L4/L3O ratio was higher in condensed "muscle" type mitochondria (heart ventricle, skeletal muscle, ratios 4;–7) than in orthodox "liver" type mitochondria (liver, smooth muscle, heart auricular appendage, H9c2 myoblasts, ratios 0.4;–3), suggesting that the L4/L3O proportion is important for cristae membrane structure.

We concluded that the L4/L3O ratio is a tissue-specific variable that may change in the presence of mitochondrial disease. The new method is suitable to measure cardiolipin in muscle biopsies in order to estimate concentration of mitochondria.—Schlame, M., S. Shanske, S. Doty, T. König, T. Sculco, S. DiMauro, and T. J. J. Blanck. Microanalysis of cardiolipin is small biopsies including skeletal muscle from patients with mitochondrial disease. J. Lipid Res. 1999. 40: 1585;–1592.

Supplementary key words: fluorescence, lipid analysis, high performance liquid chromatography, mitochondrial DNA, neuromuscular disease


Add to CiteULike CiteULike   Add to Complore Complore   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us   Add to Digg Digg   Add to Reddit Reddit   Add to Technorati Technorati    What's this?


This article has been cited by other articles:


Home page
Am. J. Physiol. Cell Physiol.Home page
A. J. Chicco and G. C. Sparagna
Role of cardiolipin alterations in mitochondrial dysfunction and disease
Am J Physiol Cell Physiol, January 1, 2007; 292(1): C33 - C44.
[Abstract] [Full Text] [PDF]


Home page
J. Biol. Chem.Home page
Y. Xu, A. Malhotra, M. Ren, and M. Schlame
The Enzymatic Function of Tafazzin
J. Biol. Chem., December 22, 2006; 281(51): 39217 - 39224.
[Abstract] [Full Text] [PDF]


Home page
Proc. Natl. Acad. Sci. USAHome page
Y. Xu, M. Condell, H. Plesken, I. Edelman-Novemsky, J. Ma, M. Ren, and M. Schlame
A Drosophila model of Barth syndrome
PNAS, August 1, 2006; 103(31): 11584 - 11588.
[Abstract] [Full Text] [PDF]


Home page
J. Gerontol. A Biol. Sci. Med. Sci.Home page
E. V. Menshikova, V. B. Ritov, L. Fairfull, R. E. Ferrell, D. E. Kelley, and B. H. Goodpaster
Effects of exercise on mitochondrial content and function in aging human skeletal muscle.
J. Gerontol. A Biol. Sci. Med. Sci., June 1, 2006; 61(6): 534 - 540.
[Abstract] [Full Text] [PDF]


Home page
J. Lipid Res.Home page
G. C. Sparagna, C. A. Johnson, S. A. McCune, R. L. Moore, and R. C. Murphy
Quantitation of cardiolipin molecular species in spontaneously hypertensive heart failure rats using electrospray ionization mass spectrometry
J. Lipid Res., June 1, 2005; 46(6): 1196 - 1204.
[Abstract] [Full Text] [PDF]


Home page
J. Biol. Chem.Home page
Y. Xu, R. I. Kelley, T. J. J. Blanck, and M. Schlame
Remodeling of Cardiolipin by Phospholipid Transacylation
J. Biol. Chem., December 19, 2003; 278(51): 51380 - 51385.
[Abstract] [Full Text] [PDF]


Home page
J Am Coll CardiolHome page
M. Schlame, R. I. Kelley, A. Feigenbaum, J. A. Towbin, P. M. Heerdt, T. Schieble, R. J. A. Wanders, S. DiMauro, and T. J. J. Blanck
Phospholipid abnormalities in children with Barth syndrome
J. Am. Coll. Cardiol., December 3, 2003; 42(11): 1994 - 1999.
[Abstract] [Full Text] [PDF]


Home page
Clin. Chem.Home page
F. Valianpour, R. J.A. Wanders, P. G. Barth, H. Overmars, and A. H. van Gennip
Quantitative and Compositional Study of Cardiolipin in Platelets by Electrospray Ionization Mass Spectrometry: Application for the Identification of Barth Syndrome Patients
Clin. Chem., September 1, 2002; 48(9): 1390 - 1397.
[Abstract] [Full Text] [PDF]


Home page
Ann. Thorac. Surg.Home page
P. M. Heerdt, M. Schlame, R. Jehle, A. Barbone, D. Burkhoff, and T. J.J. Blanck
Disease-specific remodeling of cardiac mitochondria after a left ventricular assist device
Ann. Thorac. Surg., April 1, 2002; 73(4): 1216 - 1221.
[Abstract] [Full Text] [PDF]


Home page
J. Biol. Chem.Home page
F. Jiang, M. T. Ryan, M. Schlame, M. Zhao, Z. Gu, M. Klingenberg, N. Pfanner, and M. L. Greenberg
Absence of Cardiolipin in the crd1 Null Mutant Results in Decreased Mitochondrial Membrane Potential and Reduced Mitochondrial Function
J. Biol. Chem., July 14, 2000; 275(29): 22387 - 22394.
[Abstract] [Full Text] [PDF]




HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
 All ASBMB Journals   Journal of Biological Chemistry 
 Molecular and Cellular Proteomics   ASBMB Today 
Copyright © 1999 by the American Society for Biochemistry and Molecular Biology.