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Chaperone-mediated autophagy components are upregulated in sporadic inclusion-body myositis muscle fibres

Autor: W.K. Engel, Mafalda Cacciottolo, Anna Nogalska, Carla D'Agostino, Valerie Askanas

Publikováno v: Neuropathology and Applied Neurobiology. 39:750-761

Aims Sporadic inclusion-body myositis (s-IBM) is an age-associated degenerative muscle disease. Characteristic features are muscle-fibre vacuolization and intramuscle-fibre accumulations of multiprotein aggregates, which may result from the demonstra

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::5255e9ec1fb894f73e885565ed35d5ff
https://doi.org/10.1111/nan.12038

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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

Autor: Anna Nogalska, Valerie Askanas, W.K. Engel, Carla D'Agostino

Publikováno v: Neuropathology and Applied Neurobiology. 37:315-320

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::d610d5afad399cabfd95497741769066
https://doi.org/10.1111/j.1365-2990.2010.01108.x

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Myostatin precursor protein is increased and associates with amyloid-? precursor protein in inclusion-body myositis culture model

Autor: Janis McFerrin, Anna Nogalska, W.K. Engel, S. Wojcik, Valerie Askanas, G. Oledzka

Publikováno v: Neuropathology and Applied Neurobiology. 33:238-242

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b2472b806808338aed86641ab6ac44c8
https://doi.org/10.1111/j.1365-2990.2006.00821.x

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Localization of survival motor neuron protein in human apoptotic-like and regenerating muscle fibers, and neuromuscular junctions

Autor: Aldobrando Broccolini, Valerie Askanas, W.K. Engel

Publikováno v: NeuroReport. 10:1637-1641

Mutations in the gene encoding survival motor neuron (SMN) protein are found in > 98% of patients with autosomal-recessive spinal muscular atrophy. We investigated the possible role of SMN in normal and abnormal human muscle by immunostaining biopsie

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::0fb06ee4f534edf4a432b344d4a27800
https://doi.org/10.1097/00001756-199906030-00003

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Tropomodulin Is Highly Concentrated at the Postsynaptic Domain of Human and Rat Neuromuscular Junctions

Autor: Mark A. Sussman, W.K. Engel, Laurence Kedes, Masako M. Bilak, Valerie Askanas

Publikováno v: Experimental Cell Research. 209:388-391

Tropomodulin is expressed in skeletal muscle and recent studies suggest that tropomodulin is associated with synaptic membranes. Therefore, we have examined neuromuscular junctions by immunofluorescence analysis for tropomodulin localization. Anti-tr

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::324977fa7336fd2b00d7220911369ea6
https://doi.org/10.1006/excr.1993.1325

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Frameshift and novel mutations in FUS in familial amyotrophic lateral sclerosis and ALS/dementia

Autor: Erdong Liu, W.K. Engel, Kreshnik B. Ahmeti, Benjamin Rix Brooks, J.G. Zheng, Nailah Siddique, Yong Shi, Jianhua Yan, Sandra Donkervoort, Teepu Siddique, W. Chen, Faisal Fecto, Yi Yang, Han Xiang Deng

Publikováno v: Neurology. 75(9)

Objective: Amyotrophic lateral sclerosis (ALS) is a progressive paralytic disorder caused by degeneration of motor neurons. Mutations in the FUS gene were identified in patients with familial ALS (FALS) and patients with sporadic ALS (SALS) from a va

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::78b49959ed2d006d44e5bd1628f43c26
https://pubmed.ncbi.nlm.nih.gov/20668259

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The multiple ADP/ATP translocase genes are differentially expressed during human muscle development

Autor: W.K. Engel, J Lunardi, Orest Hurko, Giuseppe Attardi

Publikováno v: Journal of Biological Chemistry. 267:15267-15270

The expression of the genes encoding the three isoforms of the human ADP/ATP translocase (T1, T2, and T3) has been analyzed at different stages of myogenic differentiation in an in vitro muscle cell system and compared with that in mature muscle. The

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::127314721542173daa2bcc2ab381f13a
https://doi.org/10.1016/s0021-9258(19)49526-4

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Effects of experimental spinal cord transection on substance P receptors: A quantitative autoradiography study

Autor: E.F. Hawkins, W.K. Engel, Giuseppe Vita, C.K. Haun

Publikováno v: Neuropeptides. 17:147-153

The autoradiographic localisation of Substance P (SP) receptors was investigated in the rat spinal cord following cordotomy at the thoracic 8–9 level. The binding of [ 125 I]-BH-SP was measured in discrete gray matter structures above (C6-C7 level)

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::0115fc7317a8ba0452457372ff94617e
https://doi.org/10.1016/0143-4179(90)90078-d

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