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Items: 1 to 50 of 439

1.

Retinal dystrophy in two boys with Costello syndrome due to the HRAS p.Gly13Cys mutation.

Pierpont ME, Richards M, Engel WK, Mendelsohn NJ, Summers CG.

Am J Med Genet A. 2017 May;173(5):1342-1347. doi: 10.1002/ajmg.a.38110. Epub 2017 Mar 23.

PMID:
28337834
2.

Activation of the Unfolded Protein Response in Sporadic Inclusion-Body Myositis but Not in Hereditary GNE Inclusion-Body Myopathy.

Nogalska A, D'Agostino C, Engel WK, Cacciottolo M, Asada S, Mori K, Askanas V.

J Neuropathol Exp Neurol. 2015 Jun;74(6):538-46. doi: 10.1097/NEN.0000000000000196.

3.

Diagnostic histochemistry and clinical-pathological testings as molecular pathways to pathogenesis and treatment of the ageing neuromuscular system: a personal view.

Engel WK.

Biochim Biophys Acta. 2015 Apr;1852(4):563-84. doi: 10.1016/j.bbadis.2014.11.015. Epub 2014 Nov 24. Review.

4.

Sporadic inclusion-body myositis: A degenerative muscle disease associated with aging, impaired muscle protein homeostasis and abnormal mitophagy.

Askanas V, Engel WK, Nogalska A.

Biochim Biophys Acta. 2015 Apr;1852(4):633-43. doi: 10.1016/j.bbadis.2014.09.005. Epub 2014 Sep 18. Review.

5.

Sodium phenylbutyrate reverses lysosomal dysfunction and decreases amyloid-β42 in an in vitro-model of inclusion-body myositis.

Nogalska A, D'Agostino C, Engel WK, Askanas V.

Neurobiol Dis. 2014 May;65:93-101. doi: 10.1016/j.nbd.2014.01.012. Epub 2014 Jan 25.

PMID:
24472798
6.

Dysferlin is a newly identified binding partner of AβPP and it co-aggregates with amyloid-β42 within sporadic inclusion-body myositis (s-IBM) muscle fibers.

Cacciottolo M, Nogalska A, D'Agostino C, Engel WK, Askanas V.

Acta Neuropathol. 2013 Nov;126(5):781-3. doi: 10.1007/s00401-013-1186-6. Epub 2013 Oct 4. No abstract available.

PMID:
24091414
7.

Ofatumumab for refractory opsoclonus-myoclonus syndrome following treatment of neuroblastoma.

Ketterl TG, Messinger YH, Niess DR, Gilles E, Engel WK, Perkins JL.

Pediatr Blood Cancer. 2013 Dec;60(12):E163-5. doi: 10.1002/pbc.24646. Epub 2013 Jun 29.

PMID:
23813921
8.

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

Cacciottolo M, Nogalska A, D'Agostino C, Engel WK, Askanas V.

Neuropathol Appl Neurobiol. 2013 Dec;39(7):750-61. doi: 10.1111/nan.12038.

PMID:
23452232
9.

Luminescent conjugated oligothiophenes for sensitive fluorescent assignment of protein inclusion bodies.

Klingstedt T, Blechschmidt C, Nogalska A, Prokop S, Häggqvist B, Danielsson O, Engel WK, Askanas V, Heppner FL, Nilsson KP.

Chembiochem. 2013 Mar 18;14(5):607-16. doi: 10.1002/cbic.201200731. Epub 2013 Feb 28.

10.

Pathogenic considerations in sporadic inclusion-body myositis, a degenerative muscle disease associated with aging and abnormalities of myoproteostasis.

Askanas V, Engel WK, Nogalska A.

J Neuropathol Exp Neurol. 2012 Aug;71(8):680-93. doi: 10.1097/NEN.0b013e31826183c8. Review.

PMID:
22805774
11.

Activation of the γ-secretase complex and presence of γ-secretase-activating protein may contribute to Aβ42 production in sporadic inclusion-body myositis muscle fibers.

Nogalska A, D'Agostino C, Engel WK, Askanas V.

Neurobiol Dis. 2012 Oct;48(1):141-9. doi: 10.1016/j.nbd.2012.06.008. Epub 2012 Jun 30.

PMID:
22750528
12.

Abnormalities of NBR1, a novel autophagy-associated protein, in muscle fibers of sporadic inclusion-body myositis.

D'Agostino C, Nogalska A, Cacciottolo M, Engel WK, Askanas V.

Acta Neuropathol. 2011 Nov;122(5):627-36. doi: 10.1007/s00401-011-0874-3. Epub 2011 Sep 21.

PMID:
21935636
13.

Novel demonstration of conformationally modified tau in sporadic inclusion-body myositis muscle fibers.

Nogalska A, D'Agostino C, Engel WK, Askanas V.

Neurosci Lett. 2011 Oct 10;503(3):229-33. doi: 10.1016/j.neulet.2011.08.042. Epub 2011 Aug 27.

PMID:
21896314
15.
16.

Novel demonstration of amyloid-β oligomers in sporadic inclusion-body myositis muscle fibers.

Nogalska A, D'Agostino C, Engel WK, Klein WL, Askanas V.

Acta Neuropathol. 2010 Nov;120(5):661-6. doi: 10.1007/s00401-010-0737-3. Epub 2010 Aug 15.

PMID:
20711838
17.

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

Yan J, Deng HX, Siddique N, Fecto F, Chen W, Yang Y, Liu E, Donkervoort S, Zheng JG, Shi Y, Ahmeti KB, Brooks B, Engel WK, Siddique T.

Neurology. 2010 Aug 31;75(9):807-14. doi: 10.1212/WNL.0b013e3181f07e0c. Epub 2010 Jul 28.

18.

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.

D'Agostino C, Nogalska A, Engel WK, Askanas V.

Neuropathol Appl Neurobiol. 2011 Apr;37(3):315-20. doi: 10.1111/j.1365-2990.2010.01108.x. No abstract available.

PMID:
20626631
19.

Impaired autophagy in sporadic inclusion-body myositis and in endoplasmic reticulum stress-provoked cultured human muscle fibers.

Nogalska A, D'Agostino C, Terracciano C, Engel WK, Askanas V.

Am J Pathol. 2010 Sep;177(3):1377-87. doi: 10.2353/ajpath.2010.100050. Epub 2010 Jul 8.

20.

Increased BACE1 mRNA and noncoding BACE1-antisense transcript in sporadic inclusion-body myositis muscle fibers--possibly caused by endoplasmic reticulum stress.

Nogalska A, Engel WK, Askanas V.

Neurosci Lett. 2010 May 3;474(3):140-143. doi: 10.1016/j.neulet.2010.03.023. Epub 2010 Mar 15.

21.
22.

Intravenous immunoglobulin in relapsing-remitting multiple sclerosis: a dose-finding trial.

Engel WK.

Neurology. 2009 Sep 29;73(13):1077; author reply 1077-8. doi: 10.1212/WNL.0b013e3181ab6b81. No abstract available.

PMID:
19786702
23.
24.

Inclusion-body myositis: muscle-fiber molecular pathology and possible pathogenic significance of its similarity to Alzheimer's and Parkinson's disease brains.

Askanas V, Engel WK.

Acta Neuropathol. 2008 Dec;116(6):583-95. doi: 10.1007/s00401-008-0449-0. Epub 2008 Oct 31. Review.

25.

Decreased SIRT1 deacetylase activity in sporadic inclusion-body myositis muscle fibers.

Nogalska A, D'Agostino C, Engel WK, Davies KJ, Askanas V.

Neurobiol Aging. 2010 Sep;31(9):1637-48. doi: 10.1016/j.neurobiolaging.2008.08.021. Epub 2008 Oct 14.

PMID:
18922603
26.

In inclusion-body myositis muscle fibers Parkinson-associated DJ-1 is increased and oxidized.

Terracciano C, Nogalska A, Engel WK, Wojcik S, Askanas V.

Free Radic Biol Med. 2008 Sep 15;45(6):773-9. doi: 10.1016/j.freeradbiomed.2008.05.030. Epub 2008 Jun 14.

27.

Myostatin and its precursor protein are increased in the skeletal muscle of patients with Type-II muscle fibre atrophy.

Wójcik S, Nogalska A, Engel WK, Askanas V.

Folia Morphol (Warsz). 2008 Feb;67(1):6-12.

PMID:
18335407
28.

Inclusion-body myositis, a multifactorial muscle disease associated with aging: current concepts of pathogenesis.

Askanas V, Engel WK.

Curr Opin Rheumatol. 2007 Nov;19(6):550-9. Review.

PMID:
17917534
29.

Expression of Nogo-A in human muscle fibers is not specific for amyotrophic lateral sclerosis.

Askanas V, Wojcik S, Engel WK.

Ann Neurol. 2007 Dec;62(6):676-7; author reply 677. No abstract available.

PMID:
17894379
30.

NOGO is increased and binds to BACE1 in sporadic inclusion-body myositis and in A beta PP-overexpressing cultured human muscle fibers.

Wojcik S, Engel WK, Yan R, McFerrin J, Askanas V.

Acta Neuropathol. 2007 Nov;114(5):517-26. Epub 2007 Aug 31.

PMID:
17764014
31.

Increased expression of Noga-A in ALS muscle biopsies is not unique for this disease.

Wojcik S, Engel WK, Askanas V.

Acta Myol. 2006 Dec;25(3):116-8.

PMID:
17626519
32.

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

Wojcik S, Nogalska A, McFerrin J, Engel WK, Oledzka G, Askanas V.

Neuropathol Appl Neurobiol. 2007 Apr;33(2):238-42. No abstract available.

PMID:
17359364
33.

Endoplasmic reticulum stress induces myostatin precursor protein and NF-kappaB in cultured human muscle fibers: relevance to inclusion body myositis.

Nogalska A, Wojcik S, Engel WK, McFerrin J, Askanas V.

Exp Neurol. 2007 Apr;204(2):610-8. Epub 2006 Dec 23.

34.

AbetaPP-overexpression and proteasome inhibition increase alphaB-crystallin in cultured human muscle: relevance to inclusion-body myositis.

Wojcik S, Engel WK, McFerrin J, Paciello O, Askanas V.

Neuromuscul Disord. 2006 Dec;16(12):839-44. Epub 2006 Oct 23.

35.
36.
37.
38.

Inclusion-body myositis: clinical, diagnostic, and pathologic aspects.

Engel WK, Askanas V.

Neurology. 2006 Jan 24;66(2 Suppl 1):S20-9. Review.

PMID:
16432141
40.

Molecular pathology and pathogenesis of inclusion-body myositis.

Askanas V, Engel WK.

Microsc Res Tech. 2005 Jul;67(3-4):114-20. Review.

PMID:
16104000
41.
42.

Myostatin is increased and complexes with amyloid-beta within sporadic inclusion-body myositis muscle fibers.

Wójcik S, Engel WK, McFerrin J, Askanas V.

Acta Neuropathol. 2005 Aug;110(2):173-7. Epub 2005 Jun 28.

PMID:
15983828
43.

Mutant ubiquitin UBB+1 is accumulated in sporadic inclusion-body myositis muscle fibers.

Fratta P, Engel WK, Van Leeuwen FW, Hol EM, Vattemi G, Askanas V.

Neurology. 2004 Sep 28;63(6):1114-7.

PMID:
15452314
44.

Ophthalmic manifestations of Wolf-Hirschhorn syndrome.

Wu-Chen WY, Christiansen SP, Berry SA, Engel WK, Fray KJ, Summers CG.

J AAPOS. 2004 Aug;8(4):345-8.

PMID:
15314595
45.

Unicorns, dragons, polymyositis, and other mythical beasts.

Askanas V, Engel WK.

Neurology. 2004 Jul 27;63(2):403-4; author reply 404. No abstract available.

PMID:
15277658
47.

Endoplasmic reticulum stress and unfolded protein response in inclusion body myositis muscle.

Vattemi G, Engel WK, McFerrin J, Askanas V.

Am J Pathol. 2004 Jan;164(1):1-7.

48.

Guidelines for the diagnosis and treatment of chronic inflammatory demyelinating polyneuropathy.

Berger AR, Bradley WG, Brannagan TH, Busis NA, Cros DP, Dalakas MC, Danon MJ, Donofrio P, Engel WK, England JD, Feldman EL, Freeman RL, Kinsella LJ, Lacomis D, Latov N, Menkes DL, Sander HW, Thomas FP, Triggs WJ, Windebank AJ, Wolfe GI; Neuropathy Association, Medical Advisory Committee.

J Peripher Nerv Syst. 2003 Dec;8(4):282-4. No abstract available.

PMID:
14641652
49.
50.

Transthyretin Val122Ile, accumulated Abeta, and inclusion-body myositis aspects in cultured muscle.

Askanas V, Engel WK, McFerrin J, Vattemi G.

Neurology. 2003 Jul 22;61(2):257-60.

PMID:
12874414

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