Spectrophotometric Assay for Complex I of the Respiratory Chain in Tissue Samples and Cultured Fibroblasts | Zendy

A.J.M. Janssen | Zendy; Frans J.M. Trijbels | Zendy; R. C. A. Sengers | Zendy; Jan Smeitink | Zendy; Lambert P. van den Heuvel | Zendy; Liesbeth T. Wintjes | Zendy; Berendien J M Stoltenborg-Hogenkamp | Zendy; Richard J. Rodenburg | Zendy

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Spectrophotometric Assay for Complex I of the Respiratory Chain in Tissue Samples and Cultured Fibroblasts

Author(s) -

A.J.M. Janssen,

Frans J.M. Trijbels,

R. C. A. Sengers,

Jan Smeitink,

Lambert P. van den Heuvel,

Liesbeth T. Wintjes,

Berendien J M Stoltenborg-Hogenkamp,

Richard J. Rodenburg

Publication year - 2007

Publication title -

clinical chemistry

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 1.705

H-Index - 218

eISSN - 1530-8561

pISSN - 0009-9147

DOI - 10.1373/clinchem.2006.078873

Subject(s) - rotenone , respiratory chain , coenzyme q – cytochrome c reductase , chemistry , mitochondrial respiratory chain , bovine serum albumin , mitochondrion , chromatography , biochemistry , solubility , organic chemistry , cytochrome c

A reliable and sensitive complex I assay is an essential tool for the diagnosis of mitochondrial disorders, but current spectrophotometric assays suffer from low sensitivity, low specificity, or both. This deficiency is mainly due to the poor solubility of coenzyme-Q analogs and reaction mixture turbidity caused by the relatively high concentrations of tissue extract that are often required to measure complex I.

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