Immunolocalization of heat shock proteins in ragged-red fibers of patients with mitochondrial encephalomyopathies

Marco Sparaco; Gorazd Rosoklija; Kurenai Tanji; Monica Sciacco; Norman Latov; Salvatore DiMauro; Eduardo Bnilla

doi:10.1016/0960-8966(93)90044-K

Immunolocalization of heat shock proteins in ragged-red fibers of patients with mitochondrial encephalomyopathies

Marco Sparaco, Gorazd Rosoklija, Kurenai Tanji, Monica Sciacco, Norman Latov, Salvatore DiMauro, Eduardo Bnilla

Fondazione IRCCS Ca' Granda- Ospedale Maggiore Policlinico

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

Abstract

Monoclonal antibodies against the 60 kDa heat shock protein (HSP-60) and against ubiquitin (UB) were used to study the expression of these proteins in muscle samples from patients with qualitative and quantitative alterations of mitochondrial DNA (mtDNA). We found an enhanced expression of HSP-60 and UB that was preferentially localized in ragged-red fibers (RRFs). HSP-60 may act as a protein repair enzyme catalyzing the refolding of misfolded proteins in the matrix of mitochondria of RRFs. On the other hand, UB could promote the elimination of abnormal proteins by its covalent interaction to substrates.

Original language	English
Pages (from-to)	71-76
Number of pages	6
Journal	Neuromuscular Disorders
Volume	3
Issue number	1
DOIs	https://doi.org/10.1016/0960-8966(93)90044-K
Publication status	Published - 1993

Keywords

heat shock proteins
Ragged-red fibers
stress response

ASJC Scopus subject areas

Clinical Neurology
Pediatrics, Perinatology, and Child Health
Developmental Neuroscience
Neurology

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abstract = "Monoclonal antibodies against the 60 kDa heat shock protein (HSP-60) and against ubiquitin (UB) were used to study the expression of these proteins in muscle samples from patients with qualitative and quantitative alterations of mitochondrial DNA (mtDNA). We found an enhanced expression of HSP-60 and UB that was preferentially localized in ragged-red fibers (RRFs). HSP-60 may act as a protein repair enzyme catalyzing the refolding of misfolded proteins in the matrix of mitochondria of RRFs. On the other hand, UB could promote the elimination of abnormal proteins by its covalent interaction to substrates.",

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T1 - Immunolocalization of heat shock proteins in ragged-red fibers of patients with mitochondrial encephalomyopathies

AU - Sparaco, Marco

AU - Rosoklija, Gorazd

AU - Tanji, Kurenai

AU - Sciacco, Monica

AU - Latov, Norman

AU - DiMauro, Salvatore

AU - Bnilla, Eduardo

PY - 1993

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AB - Monoclonal antibodies against the 60 kDa heat shock protein (HSP-60) and against ubiquitin (UB) were used to study the expression of these proteins in muscle samples from patients with qualitative and quantitative alterations of mitochondrial DNA (mtDNA). We found an enhanced expression of HSP-60 and UB that was preferentially localized in ragged-red fibers (RRFs). HSP-60 may act as a protein repair enzyme catalyzing the refolding of misfolded proteins in the matrix of mitochondria of RRFs. On the other hand, UB could promote the elimination of abnormal proteins by its covalent interaction to substrates.

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KW - Ragged-red fibers

KW - stress response

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Immunolocalization of heat shock proteins in ragged-red fibers of patients with mitochondrial encephalomyopathies

Abstract

Keywords

ASJC Scopus subject areas

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