Cytochrome C Oxidase Deficiency and Neuronal Involvement in Menkes' Kinky Hair Disease: Immunohistochemical Study

Marco Sparaco; Asao Hirano; Micho Hirano; Salvatore DiMauro; Eduardo Bonilla

doi:10.1111/j.1750-3639.1993.tb00762.x

Cytochrome C Oxidase Deficiency and Neuronal Involvement in Menkes' Kinky Hair Disease: Immunohistochemical Study

Marco Sparaco, Asao Hirano, Micho Hirano, Salvatore DiMauro, Eduardo Bonilla

Pathology

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

26 Scopus citations

Abstract

Antibodies against subunits II and IV of cytochrome c oxidase (COX) and against complex III of the respiratory chain were used to study the expression of these proteins in the cerebellum, spinal cord, and other regions of the central nervous system in an autoptic case of Menkes' kinky hair disease (MKHD). We found a reduced expression of COX subunits in all examined areas whereas staining for complex III appeared normal. Immunostaining was altered in morphologically well‐preserved neurons, suggesting that COX deficiency may have a pathogenetic role in the neuronal degeneration of MKHD.

Original language	English (US)
Pages (from-to)	349-354
Number of pages	6
Journal	Brain Pathology
Volume	3
Issue number	4
DOIs	https://doi.org/10.1111/j.1750-3639.1993.tb00762.x
State	Published - Oct 1993

ASJC Scopus subject areas

General Neuroscience
Pathology and Forensic Medicine
Clinical Neurology

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abstract = "Antibodies against subunits II and IV of cytochrome c oxidase (COX) and against complex III of the respiratory chain were used to study the expression of these proteins in the cerebellum, spinal cord, and other regions of the central nervous system in an autoptic case of Menkes' kinky hair disease (MKHD). We found a reduced expression of COX subunits in all examined areas whereas staining for complex III appeared normal. Immunostaining was altered in morphologically well‐preserved neurons, suggesting that COX deficiency may have a pathogenetic role in the neuronal degeneration of MKHD.",

author = "Marco Sparaco and Asao Hirano and Micho Hirano and Salvatore DiMauro and Eduardo Bonilla",

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AU - Hirano, Micho

AU - DiMauro, Salvatore

AU - Bonilla, Eduardo

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AB - Antibodies against subunits II and IV of cytochrome c oxidase (COX) and against complex III of the respiratory chain were used to study the expression of these proteins in the cerebellum, spinal cord, and other regions of the central nervous system in an autoptic case of Menkes' kinky hair disease (MKHD). We found a reduced expression of COX subunits in all examined areas whereas staining for complex III appeared normal. Immunostaining was altered in morphologically well‐preserved neurons, suggesting that COX deficiency may have a pathogenetic role in the neuronal degeneration of MKHD.

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Cytochrome C Oxidase Deficiency and Neuronal Involvement in Menkes' Kinky Hair Disease: Immunohistochemical Study

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