Combined deficiencies of the pyruvate dehydrogenase complex and enzymes of the respiratory chain in mitochondrial myopathies

W. Sperl; W. Ruitenbeek; R. C. A. Sengers; J. M. F. Trijbels; H. Bentlage; J. E. Wraith; C. Heilmann; S. Stöckler; C. Binder; G. -C. Korenke; F. Hanefeld

doi:10.1007/bf01954382

Combined deficiencies of the pyruvate dehydrogenase complex and enzymes of the respiratory chain in mitochondrial myopathies

European Journal of Pediatrics ◽

10.1007/bf01954382 ◽

1992 ◽

Vol 151 (3) ◽

pp. 192-195 ◽

Cited By ~ 15

Author(s):

W. Sperl ◽

W. Ruitenbeek ◽

R. C. A. Sengers ◽

J. M. F. Trijbels ◽

H. Bentlage ◽

...

Keyword(s):

Respiratory Chain ◽

Pyruvate Dehydrogenase ◽

Pyruvate Dehydrogenase Complex ◽

Mitochondrial Myopathies ◽

Dehydrogenase Complex

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Dual Inhibition of Pyruvate Dehydrogenase Complex and Respiratory Chain Complex Induces Apoptosis by a Mitochondria‐Targeted Fluorescent Organic Arsenical in vitro and in vivo

ChemMedChem ◽

10.1002/cmdc.201900686 ◽

2020 ◽

Vol 15 (6) ◽

pp. 552-558

Author(s):

Yu‐Jiao Liu ◽

Xiao‐Yang Fan ◽

Dong‐Dong Zhang ◽

Yin‐Zheng Xia ◽

Yan‐Jun Hu ◽

...

Keyword(s):

Respiratory Chain ◽

Pyruvate Dehydrogenase ◽

Pyruvate Dehydrogenase Complex ◽

Chain Complex ◽

Respiratory Chain Complex ◽

Dual Inhibition ◽

Dehydrogenase Complex

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P038 Combined defects of the pyruvate dehydrogenase complex and respiratory chain responsive to ubiquinone and riboflavin

European Journal of Paediatric Neurology ◽

10.1016/s1090-3798(09)70096-0 ◽

2009 ◽

Vol 13 ◽

pp. S33

Author(s):

J. Ng ◽

M.J. Sharrard ◽

S.E. Olpin ◽

N. Athiraman ◽

S.R. Mordekar

Keyword(s):

Respiratory Chain ◽

Pyruvate Dehydrogenase ◽

Pyruvate Dehydrogenase Complex ◽

Combined Defects ◽

Dehydrogenase Complex

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Pyruvate dehydrogenase complex deficiency and altered respiratory chain function in a patient with Kearns–Sayre/MELAS overlap syndrome and A3243G mtDNA mutation

Journal of the Neurological Sciences ◽

10.1016/s0022-510x(98)00068-9 ◽

1998 ◽

Vol 157 (2) ◽

pp. 206-213 ◽

Cited By ~ 17

Author(s):

Ekkehard Wilichowski ◽

Georg Christoph Korenke ◽

Wim Ruitenbeek ◽

Linda De Meirleir ◽

Angela Hagendorff ◽

...

Keyword(s):

Respiratory Chain ◽

Pyruvate Dehydrogenase ◽

Pyruvate Dehydrogenase Complex ◽

Overlap Syndrome ◽

Mtdna Mutation ◽

Complex Deficiency ◽

Pyruvate Dehydrogenase Complex Deficiency ◽

Dehydrogenase Complex

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Defects in the mitochondrial energy metabolism outside the respiratory chain and the pyruvate dehydrogenase complex

Detection of Mitochondrial Diseases ◽

10.1007/978-1-4615-6111-8_38 ◽

1997 ◽

pp. 243-247

Author(s):

Frans J. M. Trijbels ◽

Wim Ruitenbeek ◽

Marjan Huizing ◽

Udo Wendel ◽

Jan A. M. Smeitink ◽

...

Keyword(s):

Energy Metabolism ◽

Respiratory Chain ◽

Pyruvate Dehydrogenase ◽

Pyruvate Dehydrogenase Complex ◽

Mitochondrial Energy Metabolism ◽

Dehydrogenase Complex

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Faculty Opinions recommendation of Molecular architecture and mechanism of an icosahedral pyruvate dehydrogenase complex: a multifunctional catalytic machine.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.1008259.174909 ◽

2003 ◽

Author(s):

James K Stoops

Keyword(s):

Pyruvate Dehydrogenase ◽

Pyruvate Dehydrogenase Complex ◽

Molecular Architecture ◽

Dehydrogenase Complex

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Faculty Opinions recommendation of The malaria parasite Plasmodium falciparum has only one pyruvate dehydrogenase complex, which is located in the apicoplast.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.1023299.269657 ◽

2005 ◽

Author(s):

Jurgen Soll

Keyword(s):

Plasmodium Falciparum ◽

Pyruvate Dehydrogenase ◽

Malaria Parasite ◽

Pyruvate Dehydrogenase Complex ◽

Parasite Plasmodium ◽

Parasite Plasmodium Falciparum ◽

Dehydrogenase Complex

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The Pyruvate Dehydrogenase Complex as a Target for Gene Therapy

Current Gene Therapy ◽

10.2174/1566523034578320 ◽

2003 ◽

Vol 3 (3) ◽

pp. 239-245 ◽

Cited By ~ 15

Author(s):

Peter Stacpoole ◽

Renius Owen ◽

Terence Flotte

Keyword(s):

Gene Therapy ◽

Pyruvate Dehydrogenase ◽

Pyruvate Dehydrogenase Complex ◽

Dehydrogenase Complex

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Pyruvate Dehydrogenase Complex

RCSB Protein Data Bank ◽

10.2210/rcsb_pdb/mom_2012_9 ◽

2012 ◽

Author(s):

D.S. Goodsell

Keyword(s):

Pyruvate Dehydrogenase ◽

Pyruvate Dehydrogenase Complex ◽

Dehydrogenase Complex

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Amidination of pyruvate dehydrogenase complex of Escherichia coli under denaturing conditions

Biochemical Journal ◽

10.1042/bj1770136 ◽

1979 ◽

Vol 177 (1) ◽

pp. 136-137 ◽

Cited By ~ 9

Author(s):

G Hale ◽

E A Hooper ◽

R N Perham

Keyword(s):

Escherichia Coli ◽

Pyruvate Dehydrogenase ◽

Pyruvate Dehydrogenase Complex ◽

Dehydrogenase Complex

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Three-dimensional structure of the truncated core of the Saccharomyces cerevisiae pyruvate dehydrogenase complex determined from negative stain and cryoelectron microscopy images.

Journal of Biological Chemistry ◽

10.1016/s0021-9258(18)35830-7 ◽

1992 ◽

Vol 267 (34) ◽

pp. 24769-24775

Author(s):

J.K. Stoops ◽

T.S. Baker ◽

J.P. Schroeter ◽

S.J. Kolodziej ◽

X.D. Niu ◽

...

Keyword(s):

Saccharomyces Cerevisiae ◽

Pyruvate Dehydrogenase ◽

Pyruvate Dehydrogenase Complex ◽

Three Dimensional ◽

Dimensional Structure ◽

Cryoelectron Microscopy ◽

Three Dimensional Structure ◽

Negative Stain ◽

Microscopy Images ◽

Dehydrogenase Complex

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