Expression of the lysosome-associated membrane proteins in myopathies with rimmed vacuoles

Y. Tsuruta, A. Furuta, K. Furuta, T. Yamada, Jun-Ichi Kira, Toru Iwaki

Research output: Contribution to journalArticle

23 Citations (Scopus)

Abstract

Lysosome-associated membrane proteins (LAMPs) are structural glycoproteins located on the lysosomal membrane and are thought to have an important role in protein degradation. Increased lysosomal activity is associated with the formation of rimmed vacuoles, which are observed in various muscle disorders such as inclusion body myositis (IBM) and distal myopathy with rimmed vacuole (DMRV). In the present study, we examined LAMP-1 and LAMP-2 in biopsied muscle specimens from four cases of sporadic IBM and two of DMRV, as well as six of myopathies without rimmed vacuoles. In all cases of IBM and DMRV, immunohistochemistry showed accumulation of LAMPs in the rimmed vacuoles and the subsarcolemmal portion of the vacuolated fibers. Immunoreactivities of LAMPs in the vacuolated fibers were often associated with those of cathepsin D; however, cathepsin D was not expressed on some LAMP-positive fibers. Further, atrophic muscle fibers were sometimes positive for LAMPs expression. These findings were more prominent in LAMP-2 than in LAMP-1. Thus, LAMP-2 may play an important role in the increased protein degradation in diseased muscle fibers. The increased expression of LAMPs in the vacuolated muscle fibers may be associated with the formation of rimmed vacuoles in IBM and DMRV.

Original languageEnglish
Pages (from-to)579-584
Number of pages6
JournalActa neuropathologica
Volume101
Issue number6
DOIs
Publication statusPublished - Jan 1 2001

Fingerprint

Lysosome-Associated Membrane Glycoproteins
Inclusion Body Myositis
Vacuoles
Muscles
Cathepsin D
Proteolysis
Nonaka type Distal myopathy
Muscular Diseases

All Science Journal Classification (ASJC) codes

  • Pathology and Forensic Medicine
  • Clinical Neurology
  • Cellular and Molecular Neuroscience

Cite this

Expression of the lysosome-associated membrane proteins in myopathies with rimmed vacuoles. / Tsuruta, Y.; Furuta, A.; Furuta, K.; Yamada, T.; Kira, Jun-Ichi; Iwaki, Toru.

In: Acta neuropathologica, Vol. 101, No. 6, 01.01.2001, p. 579-584.

Research output: Contribution to journalArticle

Tsuruta, Y. ; Furuta, A. ; Furuta, K. ; Yamada, T. ; Kira, Jun-Ichi ; Iwaki, Toru. / Expression of the lysosome-associated membrane proteins in myopathies with rimmed vacuoles. In: Acta neuropathologica. 2001 ; Vol. 101, No. 6. pp. 579-584.
@article{24851d5126ee4672b9f7eadffae80850,
title = "Expression of the lysosome-associated membrane proteins in myopathies with rimmed vacuoles",
abstract = "Lysosome-associated membrane proteins (LAMPs) are structural glycoproteins located on the lysosomal membrane and are thought to have an important role in protein degradation. Increased lysosomal activity is associated with the formation of rimmed vacuoles, which are observed in various muscle disorders such as inclusion body myositis (IBM) and distal myopathy with rimmed vacuole (DMRV). In the present study, we examined LAMP-1 and LAMP-2 in biopsied muscle specimens from four cases of sporadic IBM and two of DMRV, as well as six of myopathies without rimmed vacuoles. In all cases of IBM and DMRV, immunohistochemistry showed accumulation of LAMPs in the rimmed vacuoles and the subsarcolemmal portion of the vacuolated fibers. Immunoreactivities of LAMPs in the vacuolated fibers were often associated with those of cathepsin D; however, cathepsin D was not expressed on some LAMP-positive fibers. Further, atrophic muscle fibers were sometimes positive for LAMPs expression. These findings were more prominent in LAMP-2 than in LAMP-1. Thus, LAMP-2 may play an important role in the increased protein degradation in diseased muscle fibers. The increased expression of LAMPs in the vacuolated muscle fibers may be associated with the formation of rimmed vacuoles in IBM and DMRV.",
author = "Y. Tsuruta and A. Furuta and K. Furuta and T. Yamada and Jun-Ichi Kira and Toru Iwaki",
year = "2001",
month = "1",
day = "1",
doi = "10.1007/s004010000329",
language = "English",
volume = "101",
pages = "579--584",
journal = "Acta Neuropathologica",
issn = "0001-6322",
publisher = "Springer Verlag",
number = "6",

}

TY - JOUR

T1 - Expression of the lysosome-associated membrane proteins in myopathies with rimmed vacuoles

AU - Tsuruta, Y.

AU - Furuta, A.

AU - Furuta, K.

AU - Yamada, T.

AU - Kira, Jun-Ichi

AU - Iwaki, Toru

PY - 2001/1/1

Y1 - 2001/1/1

N2 - Lysosome-associated membrane proteins (LAMPs) are structural glycoproteins located on the lysosomal membrane and are thought to have an important role in protein degradation. Increased lysosomal activity is associated with the formation of rimmed vacuoles, which are observed in various muscle disorders such as inclusion body myositis (IBM) and distal myopathy with rimmed vacuole (DMRV). In the present study, we examined LAMP-1 and LAMP-2 in biopsied muscle specimens from four cases of sporadic IBM and two of DMRV, as well as six of myopathies without rimmed vacuoles. In all cases of IBM and DMRV, immunohistochemistry showed accumulation of LAMPs in the rimmed vacuoles and the subsarcolemmal portion of the vacuolated fibers. Immunoreactivities of LAMPs in the vacuolated fibers were often associated with those of cathepsin D; however, cathepsin D was not expressed on some LAMP-positive fibers. Further, atrophic muscle fibers were sometimes positive for LAMPs expression. These findings were more prominent in LAMP-2 than in LAMP-1. Thus, LAMP-2 may play an important role in the increased protein degradation in diseased muscle fibers. The increased expression of LAMPs in the vacuolated muscle fibers may be associated with the formation of rimmed vacuoles in IBM and DMRV.

AB - Lysosome-associated membrane proteins (LAMPs) are structural glycoproteins located on the lysosomal membrane and are thought to have an important role in protein degradation. Increased lysosomal activity is associated with the formation of rimmed vacuoles, which are observed in various muscle disorders such as inclusion body myositis (IBM) and distal myopathy with rimmed vacuole (DMRV). In the present study, we examined LAMP-1 and LAMP-2 in biopsied muscle specimens from four cases of sporadic IBM and two of DMRV, as well as six of myopathies without rimmed vacuoles. In all cases of IBM and DMRV, immunohistochemistry showed accumulation of LAMPs in the rimmed vacuoles and the subsarcolemmal portion of the vacuolated fibers. Immunoreactivities of LAMPs in the vacuolated fibers were often associated with those of cathepsin D; however, cathepsin D was not expressed on some LAMP-positive fibers. Further, atrophic muscle fibers were sometimes positive for LAMPs expression. These findings were more prominent in LAMP-2 than in LAMP-1. Thus, LAMP-2 may play an important role in the increased protein degradation in diseased muscle fibers. The increased expression of LAMPs in the vacuolated muscle fibers may be associated with the formation of rimmed vacuoles in IBM and DMRV.

UR - http://www.scopus.com/inward/record.url?scp=0034919522&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0034919522&partnerID=8YFLogxK

U2 - 10.1007/s004010000329

DO - 10.1007/s004010000329

M3 - Article

C2 - 11515786

AN - SCOPUS:0034919522

VL - 101

SP - 579

EP - 584

JO - Acta Neuropathologica

JF - Acta Neuropathologica

SN - 0001-6322

IS - 6

ER -