TY - JOUR
T1 - PAIRED HELICAL FILAMENTS OF INCLUSION-BODY MYOSITIS CONTAIN RNA AND SURVIVAL MOTOR NEURON PROTEIN
AU - Broccolini, Aldobrando
AU - Engel, Wk
AU - Alvarez, Rb
AU - Askanas, V.
PY - 2000
Y1 - 2000
N2 - Sporadic inclusion-body myositis (s-IBM) is the most common progressive muscle disease of older persons. Pathologically, the muscle biopsy manifests various degrees of inflammation and specific vacuolar degeneration of muscle fibers characterized by paired helical filaments (PHFs) composed of phosphorylated tau. IBM vacuolated fibers also contain accumulations of several other Alzheimer-characteristic proteins. Molecular mechanisms leading to formation of the PHFs and accumulations of proteins in IBM muscle are not known. We report that the abnormal muscle fibers of IBM contained (i) acridine-orange-positive RNA inclusions that colocalized with the immunoreactivity of phosphorylated tau and (ii) survival motor neuron protein immunoreactive inclusions, which by immuno-electron microscopy were confined to paired helical filaments. This study demonstrates two novel components of the IBM paired helical filaments, which may lead to better understanding of their pathogenesis.
AB - Sporadic inclusion-body myositis (s-IBM) is the most common progressive muscle disease of older persons. Pathologically, the muscle biopsy manifests various degrees of inflammation and specific vacuolar degeneration of muscle fibers characterized by paired helical filaments (PHFs) composed of phosphorylated tau. IBM vacuolated fibers also contain accumulations of several other Alzheimer-characteristic proteins. Molecular mechanisms leading to formation of the PHFs and accumulations of proteins in IBM muscle are not known. We report that the abnormal muscle fibers of IBM contained (i) acridine-orange-positive RNA inclusions that colocalized with the immunoreactivity of phosphorylated tau and (ii) survival motor neuron protein immunoreactive inclusions, which by immuno-electron microscopy were confined to paired helical filaments. This study demonstrates two novel components of the IBM paired helical filaments, which may lead to better understanding of their pathogenesis.
KW - Acridine Orange
KW - Antibodies, Monoclonal
KW - Cyclic AMP Response Element-Binding Protein
KW - Fluorescent Dyes
KW - Humans
KW - Immunohistochemistry
KW - Muscles
KW - Myositis, Inclusion Body
KW - Nerve Tissue Proteins
KW - Neuropil Threads
KW - RNA
KW - RNA-Binding Proteins
KW - SMN Complex Proteins
KW - Staining and Labeling
KW - Tissue Distribution
KW - Acridine Orange
KW - Antibodies, Monoclonal
KW - Cyclic AMP Response Element-Binding Protein
KW - Fluorescent Dyes
KW - Humans
KW - Immunohistochemistry
KW - Muscles
KW - Myositis, Inclusion Body
KW - Nerve Tissue Proteins
KW - Neuropil Threads
KW - RNA
KW - RNA-Binding Proteins
KW - SMN Complex Proteins
KW - Staining and Labeling
KW - Tissue Distribution
UR - http://hdl.handle.net/10807/37339
U2 - 10.1016/S0002-9440(10)64983-8
DO - 10.1016/S0002-9440(10)64983-8
M3 - Article
SN - 0002-9440
VL - 156
SP - 1151
EP - 1155
JO - THE AMERICAN JOURNAL OF PATHOLOGY
JF - THE AMERICAN JOURNAL OF PATHOLOGY
ER -