Changes in mitochondrial complex I activity and coenzyme Q binding site in Leber's hereditary optic neuropathy (LHON)

A. Ghelli; M. Degli Esposti; V. Carelli; G. Lenaz

doi:10.1016/S0098-2997(97)00028-9

Changes in mitochondrial complex I activity and coenzyme Q binding site in Leber's hereditary optic neuropathy (LHON)

A. Ghelli, M. Degli Esposti, V. Carelli, G. Lenaz

Istituto Scienze Neurologiche

Research output: Contribution to journal › Article › peer-review

Abstract

The complex I function in sub-mitochondrial particles was studied in platelets from patients and healthy carriers with 11778/ND4 or 3460/ND1 mtDNA point mutations associated with LHON. Both 11778/ND4 and 3460/ND1 mutations induced rotenone resistance and 11778/ND4 showed an increased K(m) for ubiquinol-2 with respect to the control group. It was concluded that even with different pathogenic mechanisms both mutations affect the quinone binding site of complex I.

Original language	English
Journal	Molecular Aspects of Medicine
Volume	18
Issue number	SUPPL.
DOIs	https://doi.org/10.1016/S0098-2997(97)00028-9
Publication status	Published - 1997

Keywords

Complex I
LHON
mtDNA mutations
Platelets
Rotenone

ASJC Scopus subject areas

Biochemistry
Molecular Biology
Molecular Medicine

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10.1016/S0098-2997(97)00028-9

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title = "Changes in mitochondrial complex I activity and coenzyme Q binding site in Leber's hereditary optic neuropathy (LHON)",

abstract = "The complex I function in sub-mitochondrial particles was studied in platelets from patients and healthy carriers with 11778/ND4 or 3460/ND1 mtDNA point mutations associated with LHON. Both 11778/ND4 and 3460/ND1 mutations induced rotenone resistance and 11778/ND4 showed an increased K(m) for ubiquinol-2 with respect to the control group. It was concluded that even with different pathogenic mechanisms both mutations affect the quinone binding site of complex I.",

keywords = "Complex I, LHON, mtDNA mutations, Platelets, Rotenone",

author = "A. Ghelli and {Degli Esposti}, M. and V. Carelli and G. Lenaz",

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AU - Ghelli, A.

AU - Degli Esposti, M.

AU - Carelli, V.

AU - Lenaz, G.

PY - 1997

Y1 - 1997

N2 - The complex I function in sub-mitochondrial particles was studied in platelets from patients and healthy carriers with 11778/ND4 or 3460/ND1 mtDNA point mutations associated with LHON. Both 11778/ND4 and 3460/ND1 mutations induced rotenone resistance and 11778/ND4 showed an increased K(m) for ubiquinol-2 with respect to the control group. It was concluded that even with different pathogenic mechanisms both mutations affect the quinone binding site of complex I.

AB - The complex I function in sub-mitochondrial particles was studied in platelets from patients and healthy carriers with 11778/ND4 or 3460/ND1 mtDNA point mutations associated with LHON. Both 11778/ND4 and 3460/ND1 mutations induced rotenone resistance and 11778/ND4 showed an increased K(m) for ubiquinol-2 with respect to the control group. It was concluded that even with different pathogenic mechanisms both mutations affect the quinone binding site of complex I.

KW - Complex I

KW - LHON

KW - mtDNA mutations

KW - Platelets

KW - Rotenone

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ER -

Changes in mitochondrial complex I activity and coenzyme Q binding site in Leber's hereditary optic neuropathy (LHON)

Abstract

Keywords

ASJC Scopus subject areas

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