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Human-to-mouse prion-like propagation of mutant huntingtin protein
Springer Science and Business Media LLC - Tập 132 - Trang 577-592 - 2016
Iksoo Jeon, Francesca Cicchetti, Giulia Cisbani, Suji Lee, Endan Li, Jiwoo Bae, Nayeon Lee, Ling Li, Wooseok Im, Manho Kim, Hyun Sook Kim, Seung-Hun Oh, Tae-Aug Kim, Jung Jae Ko, Benoit Aubé, Abid Oueslati, Yun Joong Kim, Jihwan Song
Huntington’s disease (HD) is an autosomal dominant neurodegenerative disorder of the central nervous system (CNS) that is defined by a CAG expansion in exon 1 of the huntingtin gene leading to the production of mutant huntingtin (mHtt). To date, the disease pathophysiology has been thought to be primarily driven by cell-autonomous mechanisms, but, here, we demonstrate that fibroblasts derived from...... hiện toàn bộ
Pituitary adenomas: Immunohistology and ultrastructural analysis of 118 tumors
Springer Science and Business Media LLC - Tập 62 - Trang 1-14 - 1983
M. M. Esiri, C. B. T. Adams, C. Burke, R. Underdown
An analysis is presented of the immunohistological and ultrastructural features in a series of 118 surgically removed pituitary adenomas all of which were studied immunohistologically using antisera to growth hormone (GH), prolactin (PRL) ACTH, βFSH, βLH and βTSH, and 75 of which were studied ultrastructurally. Results were analysed according to the mode of presentation of patients. Forty-one (35%...... hiện toàn bộ
Effect of arachidonic acid on cultured cerebromicrovascular endothelium: permeability, lipid peroxidation and membrane “fluidity”
Springer Science and Business Media LLC - Tập 78 - Trang 310-316 - 1989
A. Villacara, M. Spatz, R. F. Dodson, C. Corn, J. Bembry
The relationship of free arachidonic acid (AA) to cellular permeability, lipid peroxidation and physical state “fluidity” of the membrane was investigated in cultured endothelial cells (EC) dissociated from cerebral microvessels of rats. The results demonstrate that AA can induce a reversible alteration of endothelial permeability to trypan blue albumin (TBA). Exposure of EC to AA increases membra...... hiện toàn bộ
Amyloid plaque formation precedes dendritic spine loss
Springer Science and Business Media LLC - Tập 124 Số 6 - Trang 797-807 - 2012
Tobias Bittner, Steffen Burgold, Mario M. Dorostkar, Martin Fuhrmann, Bettina M. Wegenast‐Braun, Boris Schmidt, Hans A. Kretzschmar, Jochen Herms
Immunohistochemical studies on cellular character of microtumors induced by ethylnitrosourea in the rat brain utilizing anti-leu 7 and anti-glial fibrillary acidic protein antibodies
Springer Science and Business Media LLC - - 1985
T. Yoshino, M. Motoi, K. Ogawa
To clarify the chronologic changes in the cellular morphology of ENU-induced rat brain tumors, microtumors in the early stage were examined ummunohistochemically in comparison with macrotumors in the advanced stage. The tumor cells composing microtumors were negative for glial fibrillary acidic protein (GFAP), a specific marker of astrocylic cells, and Leu 7, a marker of oligodendrocytes, while ce...... hiện toàn bộ
Focal ischemia of the rat brain, with special reference to the influence of plasma glucose concentration
Springer Science and Business Media LLC - - 1987
Maiken Nedergaard, Nils Henrik Diemer
Mitochondrial encephalomyopathies and cytochrome c oxidase deficiency: muscle culture study
Springer Science and Business Media LLC - - 1991
Ikuya Nonaka, Yasutoshi Koga, Azusa Kikuchi, Yu‐ichi Goto
Impacts of massively parallel sequencing for genetic diagnosis of neuromuscular disorders
Springer Science and Business Media LLC - Tập 125 - Trang 173-185 - 2012
Nasim Vasli, Jocelyn Laporte
Neuromuscular disorders (NMD) such as neuropathy or myopathy are rare and often severe inherited disorders, affecting muscle and/or nerves with neonatal, childhood or adulthood onset, with considerable burden for the patients, their families and public health systems. Genetic and clinical heterogeneity, unspecific clinical features, unidentified genes and the implication of large and/or several ge...... hiện toàn bộ
Progress in investigations of sphingolipidoses
Springer Science and Business Media LLC - Tập 43 - Trang 1-18 - 1978
M. Adachi, L. Schneck, B. W. Volk
Degeneration and regeneration in teased nerve fibres
Springer Science and Business Media LLC - Tập 18 - Trang 286-298 - 1971
Darab K. Dastur, Zohra A. Razzak
The pattern of myelin degeneration and regeneration was studied in 40 nerve biopsy or necropsy specimens, using the teased fibre method, including 35 specimens of leprous neuritis, and 5 control specimens, 3 from humans and 2 from monkeys. The specimens of leprous nerves were derived from 10 lepromatous, borderline, or tuberculoid cases in an advanced polyneuritic stage. Nearly two-thirds showed W...... hiện toàn bộ
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