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 | |
| Publication status | Published - 1993 |
Fingerprint
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 journal › Article
}
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 -