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

Research output: Contribution to journalArticle

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 languageEnglish
Pages (from-to)71-76
Number of pages6
JournalNeuromuscular Disorders
Volume3
Issue number1
DOIs
Publication statusPublished - 1993

Fingerprint

Mitochondrial Encephalomyopathies
Ubiquitin
Heat-Shock Proteins
Protein Refolding
Chaperonin 60
Muscle Proteins
Mitochondrial DNA
Mitochondria
Proteins
Monoclonal Antibodies
Enzymes

Keywords

  • heat shock proteins
  • Ragged-red fibers
  • stress response

ASJC Scopus subject areas

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

Cite this

Immunolocalization of heat shock proteins in ragged-red fibers of patients with mitochondrial encephalomyopathies. / Sparaco, Marco; Rosoklija, Gorazd; Tanji, Kurenai; Sciacco, Monica; Latov, Norman; DiMauro, Salvatore; Bnilla, Eduardo.

In: Neuromuscular Disorders, Vol. 3, No. 1, 1993, p. 71-76.

Research output: Contribution to journalArticle

Sparaco, Marco ; Rosoklija, Gorazd ; Tanji, Kurenai ; Sciacco, Monica ; Latov, Norman ; DiMauro, Salvatore ; Bnilla, Eduardo. / Immunolocalization of heat shock proteins in ragged-red fibers of patients with mitochondrial encephalomyopathies. In: Neuromuscular Disorders. 1993 ; Vol. 3, No. 1. pp. 71-76.
@article{4e05331d4af745649abcd7bf6e0e51f3,
title = "Immunolocalization of heat shock proteins in ragged-red fibers of patients with mitochondrial encephalomyopathies",
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.",
keywords = "heat shock proteins, Ragged-red fibers, stress response",
author = "Marco Sparaco and Gorazd Rosoklija and Kurenai Tanji and Monica Sciacco and Norman Latov and Salvatore DiMauro and Eduardo Bnilla",
year = "1993",
doi = "10.1016/0960-8966(93)90044-K",
language = "English",
volume = "3",
pages = "71--76",
journal = "Neuromuscular Disorders",
issn = "0960-8966",
publisher = "Elsevier Ltd",
number = "1",

}

TY - JOUR

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

Y1 - 1993

N2 - 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.

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.

KW - heat shock proteins

KW - Ragged-red fibers

KW - stress response

UR - http://www.scopus.com/inward/record.url?scp=0027322582&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0027322582&partnerID=8YFLogxK

U2 - 10.1016/0960-8966(93)90044-K

DO - 10.1016/0960-8966(93)90044-K

M3 - Article

C2 - 8101114

AN - SCOPUS:0027322582

VL - 3

SP - 71

EP - 76

JO - Neuromuscular Disorders

JF - Neuromuscular Disorders

SN - 0960-8966

IS - 1

ER -