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Cultured inclusion-body myositis muscle fibers do not accumulate beta-amyloid precursor protein and can be innervated.
McFerrin J, Engel WK, Askanas V. McFerrin J, et al. Among authors: engel wk. Neurology. 1999 Dec 10;53(9):2184-7. doi: 10.1212/wnl.53.9.2184. Neurology. 1999. PMID: 10599804
Thus, factors responsible for betaAPP accumulation and denervation-like changes in s-IBM muscle biopsies are not operative in the relatively short-term, non-aged, cultured s-IBM muscle fibers....
Thus, factors responsible for betaAPP accumulation and denervation-like changes in s-IBM muscle biopsies are not operative in the relatively …
Light and electron microscopic immunolocalization of presenilin 1 in abnormal muscle fibers of patients with sporadic inclusion-body myositis and autosomal-recessive inclusion-body myopathy.
Askanas V, Engel WK, Yang CC, Alvarez RB, Lee VM, Wisniewski T. Askanas V, et al. Among authors: engel wk. Am J Pathol. 1998 Apr;152(4):889-95. Am J Pathol. 1998. PMID: 9546349 Free PMC article.
The muscle biopsy demonstrates mononuclear cell inflammation and vacuolated muscle fibers containing paired helical filaments and 6- to 10-nm fibrils, both resembling those of Alzheimer disease brain and Congo red positivity. The term hereditary inclusion-body myopathies ( …
The muscle biopsy demonstrates mononuclear cell inflammation and vacuolated muscle fibers containing paired helical filaments and 6- to 10-n …
New organotypic model to culture the entire fetal rat spinal cord.
Mariotti C, Askanas V, Engel WK. Mariotti C, et al. Among authors: engel wk. J Neurosci Methods. 1993 Jun;48(1-2):157-67. doi: 10.1016/s0165-0270(05)80018-4. J Neurosci Methods. 1993. PMID: 8377519
However, the co-cultured muscle fibers definitely influenced: (a) neurite outgrowth, (b) the expression of neurofilament proteins and (c) the ability to maintain cultured MEs long-term. This new system allows study of motor neurons and the influence on them of putative gro …
However, the co-cultured muscle fibers definitely influenced: (a) neurite outgrowth, (b) the expression of neurofilament proteins and (c) th …
Long-term treatment with glucocorticoids increases synthesis and stability of junctional acetylcholine receptors on innervated cultured human muscle.
Braun S, Askanas V, Engel WK, Ibrahim EN. Braun S, et al. Among authors: engel wk. J Neurochem. 1993 May;60(5):1929-35. doi: 10.1111/j.1471-4159.1993.tb13422.x. J Neurochem. 1993. PMID: 7682603
We have studied the effect of long-term treatment with hydrocortisone on the expression of acetylcholine receptors (AChRs) at the neuromuscular junctions of human muscle cultured in monolayer and innervated de novo by fetal rat spinal cord motoneurons. ...
We have studied the effect of long-term treatment with hydrocortisone on the expression of acetylcholine receptors (AChRs) at the neu …
Glucocorticoid increases acetylcholinesterase and organization of the postsynaptic membrane in innervated cultured human muscle.
Askanas V, McFerrin J, Park-Matsumoto YC, Lee CS, Engel WK. Askanas V, et al. Among authors: engel wk. Exp Neurol. 1992 Mar;115(3):368-75. doi: 10.1016/0014-4886(92)90201-z. Exp Neurol. 1992. PMID: 1537395
Because the increased organization of the postsynaptic folds and the increased AChE seem to be salutory effects on the NMJ of prolonged HC treatment, these changes of the NMJ itself might contribute to the long-term beneficial effect of prednisone, another glucocorticoid, …
Because the increased organization of the postsynaptic folds and the increased AChE seem to be salutory effects on the NMJ of prolonged HC t …
Different degradation rates of junctional and extrajunctional acetylcholine receptors of human muscle cultured in monolayer and innervated by fetal rat spinal cord neurons.
Braun S, Askanas V, Engel WK. Braun S, et al. Among authors: engel wk. Int J Dev Neurosci. 1992;10(1):37-44. doi: 10.1016/0736-5748(92)90005-k. Int J Dev Neurosci. 1992. PMID: 1609620
We have now studied the degradation rate of the junctional and extrajunctional AChRs, utilizing our tissue culture model, in which well-differentiated neuromuscular junctions (NMJs) form on human muscle cultured in monolayer and innervated long-term by fetal rat spinal cor …
We have now studied the degradation rate of the junctional and extrajunctional AChRs, utilizing our tissue culture model, in which well-diff …
Long-term plasmapheresis therapy is effective and safe in children with chronic relapsing dysimmune polyneuropathy.
Beydoun SR, Engel WK, Karofsky P, Schwartz MU. Beydoun SR, et al. Among authors: engel wk. Rev Neurol (Paris). 1990;146(2):123-7. Rev Neurol (Paris). 1990. PMID: 2157270
The dependence of these 2 patients on regular pheresis for the last 8-1/2 and 9 years proves its efficacy and safety in children. Long-term maintenance pheresis may be beneficial in patients with other forms of chronic dysimmune neuropathy....
The dependence of these 2 patients on regular pheresis for the last 8-1/2 and 9 years proves its efficacy and safety in children. Long-te …
Accumulation of CK-MM is impaired in innervated and contracting cultured muscle fibers of Duchenne muscular dystrophy patients.
Askanas V, Martinuzzi A, Engel WK, Kobayashi T, Stern LZ, Hsu JD. Askanas V, et al. Among authors: engel wk. Life Sci. 1987 Aug 24;41(8):927-33. doi: 10.1016/0024-3205(87)90678-3. Life Sci. 1987. PMID: 3613854
We now report that the accumulation of the muscle-"specific" isozyme of creatine kinase (CK-MM) was significantly and preferentially impaired in long-term innervated contracting muscle fibers cultured from 4 DMD patients (DMD-InnCMFs) compared to: i) their noninnervated si …
We now report that the accumulation of the muscle-"specific" isozyme of creatine kinase (CK-MM) was significantly and preferentially impaire …
Chronic relapsing (dysimmune) polyneuropathy: pathogenesis and treatment.
Dalakas MC, Engel WK. Dalakas MC, et al. Among authors: engel wk. Ann Neurol. 1981;9 Suppl:134-45. doi: 10.1002/ana.410090719. Ann Neurol. 1981. PMID: 7224612
Immunoglobulin deposition observed in sural nerve biopsies and abnormal immunoglobulin patterns in the "CSF in some cases suggest a dysimmune pathogenesis; thus the term chronic relapsing (dysimmune) polyneuropathy (CRDP) is preferred. ...
Immunoglobulin deposition observed in sural nerve biopsies and abnormal immunoglobulin patterns in the "CSF in some cases suggest a dysimmun …
Adrenomyeloneuropathy: a probable variant of adrenoleukodystrophy. I. Clinical and endocrinologic aspects.
Griffin JW, Goren E, Schaumburg H, Engel WK, Loriaux L. Griffin JW, et al. Among authors: engel wk. Neurology. 1977 Dec;27(12):1107-13. doi: 10.1212/wnl.27.12.1107. Neurology. 1977. PMID: 200861
We have studied four unrelated males with a heritable disorder that we term adrenomyeloneuropathy; limited clinical information is available on a fifth case. ...
We have studied four unrelated males with a heritable disorder that we term adrenomyeloneuropathy; limited clinical information is av …
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