Search Results - "Engel, W K"

Frameshift and novel mutations in FUS in familial amyotrophic lateral sclerosis and ALS/dementia by YAN, J, DENG, H.-X, AHMETI, K. B, BROOKS, B, ENGEL, W. K, SIDDIQUE, T, SIDDIQUE, N, FECTO, F, CHEN, W, YANG, Y, LIU, E, DONKERVOORT, S, ZHENG, J. G, SHI, Y

“…Amyotrophic lateral sclerosis (ALS) is a progressive paralytic disorder caused by degeneration of motor neurons. Mutations in the FUS gene were identified in…”
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In sporadic inclusion body myositis muscle fibres TDP-43-positive inclusions are less frequent and robust than p62 inclusions, and are not associated with paired helical filaments by D'Agostino, C., Nogalska, A., Engel, W. K., Askanas, V.

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Mutant ubiquitin UBB+1 is accumulated in sporadic inclusion-body myositis muscle fibers by FRATTA, P, ENGEL, W. K, VAN LEEUWEN, F. W, HOL, E. M, VATTEMI, G, ASKANAS, V

“…Mutant ubiquitin (UBB+1), a product of "molecular misreading," is toxic to cells because its ubiquitinated form inhibits the proteasome, contributing to…”
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Myostatin precursor protein is increased and associates with amyloid-β precursor protein in inclusion-body myositis culture model by Wojcik, S., Nogalska, A., McFerrin, J., Engel, W. K., Oledzka, G., Askanas, V.

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Transfer of β -Amyloid Precursor Protein Gene Using Adenovirus Vector Causes Mitochondrial Abnormalities in Cultured Normal Human Muscle by Askanas, V., McFerrin, J., Baqué, S., Alvarez, R. B., Sarkozi, E., Engel, W. K.

“…As in Alzheimer-disease (AD) brain, vacuolated muscle fibers of inclusion-body myositis (IBM) contain abnormally accumulated β -amyloid precursor protein (β…”
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Three lipoprotein receptors and cholesterol in inclusion-body myositis muscle by JAWORSKA-WILCZYNSKA, M, WILCZYNSKI, G. M, ENGEL, W. K, STRICKLAND, D. K, WEISGRABER, K. H, ASKANAS, V

“…An important aspect of inclusion-body myositis (IBM) vacuolated muscle fibers (VMF) is abnormal accumulation of amyloid-beta precursor protein (AbetaPP)…”
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Reversible ocular myasthenia gravis or mitochondrial myopathy from statins? by Engel, WK

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Novel Immunolocalization of α-Synuclein in Human Muscle of Inclusion-Body Myositis, Regenerating and Necrotic Muscle Fibers, and at Neuromuscular Junctions by ASKANAS, VALERIE, ENGEL, W KING, ALVAREZ, RENATE B, MCFERRIN, JANIS, BROCCOLINI, ALDOBRANDO

“…α-synuclein (α-syn) is an important component of neuronal and glial inclusions in brains of patients with several neurodegenerative disorders. Sporadic…”
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Light and electron microscopic localization of beta-amyloid protein in muscle biopsies of patients with inclusion-body myositis by Askanas, V, Engel, WK, Alvarez, RB

“…In 11 of 11 inclusion-body myositis (IBM) patients, including one hereditary case, vacuolated muscle fibers contained large and multiple small inclusions…”
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Chaperone-mediated autophagy components are upregulated in sporadic inclusion-body myositis muscle fibres by Cacciottolo, M., Nogalska, A., D'Agostino, C., Engel, W. K., Askanas, V.

“…Aims Sporadic inclusion‐body myositis (s‐IBM) is an age‐associated degenerative muscle disease. Characteristic features are muscle‐fibre vacuolization and…”
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Paired Helical Filaments of Inclusion-Body Myositis Muscle Contain RNA and Survival Motor Neuron Protein by Broccolini, Aldobrando, Engel, W. King, Alvarez, Renate B., Askanas, Valerie

“…Sporadic inclusion-body myositis (s-IBM) is the most common progressive muscle disease of older persons. Pathologically, the muscle biopsy manifests various…”
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Nitric oxide-induced oxidative stress in autosomal recessive and dominant inclusion-body myopathies by YANG, C.-C, ALVAREZ, R. B, ENGEL, W. K, HELLER, S. L, ASKANAS, V

“…Autosomal-recessive and autosomal-dominant hereditary inclusion-body myopathies are severe, progressive muscle diseases, characterized pathologically by…”
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Does Overexpression of βAPP in Aging Muscle Have a Pathogenic Role and a Relevance to Alzheimer's Disease?: Clues from Inclusion Body Myositis, Cultured Human Muscle, and Transgenic Mice by Askanas, Valerie, Engel, W. King

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beta-Amyloid precursor epitopes in muscle fibers of inclusion body myositis by Askanas, V, Alvarez, R B, Engel, W K

“…Sporadic inclusion body myositis (IBM) and hereditary inclusion body myopathy (hIBM) are severe and progressive muscle diseases, characterized pathologically…”
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Difference in Expression of Phosphorylated Tau Epitopes between Sporadic Inclusion-body Myositis and Hereditary Inclusion-body Myopathies by Mirabella, Massimiliano, Alvarez, Renate B, Bilak, Masako, Engel, W King, Askanas, Valerie

“…Sporadic inclusion-body myositis (s-IBM) and the hereditary inclusion-body myopathies (h-IBMs) are severe and progressive muscle diseases, characterized…”
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Association of Active Extracellular Signal-Regulated Protein Kinase with Paired Helical Filaments of Inclusion-Body Myositis Muscle Suggests Its Role in Inclusion-Body Myositis Tau Phosphorylation by Wilczynski, Grzegorz M., Engel, W. King, Askanas, Valerie

“…The possible role of extracellular signal-regulated kinase (ERK) in the pathogenesis of inclusion-body myositis (IBM) was investigated by immunostaining the…”
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Polyneuropathy in type 2 diabetes mellitus by Engel, WK

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Localization of survival motor neuron protein in human apoptotic-like and regenerating muscle fibers, and neuromuscular junctions by Broccolini, Aldobrando, Engel, W King, Askanas, Valerie

“…MUTATIONS in the gene encoding survival motor neuron (SMN) protein are found in > 98% of patients with autosomal-recessive spinal muscular atrophy. We…”
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Twisted tubulofilaments of inclusion body myositis muscle resemble paired helical filaments of Alzheimer brain and contain hyperphosphorylated tau by Askanas, V, Engel, WK, Bilak, M, Alvarez, RB, Selkoe, DJ

“…We immunostained muscle biopsies of 8 patients with sporadic inclusion body myositis (S-IBM), 7 patients with autosomal recessive hereditary inclusion body…”
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Cultured inclusion-body myositis muscle fibers do not accumulate β-amyloid precursor protein and can be innervated by MCFERRIN, J, ENGEL, W. K, ASKANAS, V

“…Cultured muscle fibers from patients with sporadic inclusion-body myositis (s-IBM), similar to normal control muscle fibers, 1) did not have beta-amyloid…”
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