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1Latest Papers from Table of Contents or Articles in PressH. J. Kim ; N. C. Kim ; Y. D. Wang ; E. A. Scarborough ; J. Moore ; Z. Diaz ; K. S. MacLea ; B. Freibaum ; S. Li ; A. Molliex ; A. P. Kanagaraj ; R. Carter ; K. B. Boylan ; A. M. Wojtas ; R. Rademakers ; J. L. Pinkus ; S. A. Greenberg ; J. Q. Trojanowski ; B. J. Traynor ; B. N. Smith ; S. Topp ; A. S. Gkazi ; J. Miller ; C. E. Shaw ; M. Kottlors ; J. Kirschner ; A. Pestronk ; Y. R. Li ; A. F. Ford ; A. D. Gitler ; M. Benatar ; O. D. King ; V. E. Kimonis ; E. D. Ross ; C. C. Weihl ; J. Shorter ; J. P. Taylor
Nature Publishing Group (NPG)
Published 2013Staff ViewPublication Date: 2013-03-05Publisher: Nature Publishing Group (NPG)Print ISSN: 0028-0836Electronic ISSN: 1476-4687Topics: BiologyChemistry and PharmacologyMedicineNatural Sciences in GeneralPhysicsKeywords: Amino Acid Sequence ; Amyotrophic Lateral Sclerosis/*genetics/metabolism/*pathology ; Animals ; Drosophila melanogaster/cytology/genetics/metabolism ; Female ; Frontotemporal Dementia/*genetics/metabolism/pathology ; HeLa Cells ; Heterogeneous-Nuclear Ribonucleoprotein Group A-B/*chemistry/genetics/*metabolism ; Humans ; Inclusion Bodies/genetics/metabolism/pathology ; Male ; Mice ; Molecular Sequence Data ; Muscular Dystrophies, Limb-Girdle/*genetics/metabolism/pathology ; Mutant Proteins/chemistry/*genetics/metabolism ; Mutation/*genetics ; Myositis, Inclusion Body/*genetics/metabolism/pathology ; Osteitis Deformans/*genetics/metabolism/pathology ; Peptide Termination Factors/chemistry/genetics/metabolism ; Prions/*chemistry/genetics/metabolism ; Protein Structure, Tertiary/genetics ; RNA/metabolism ; Saccharomyces cerevisiae Proteins/chemistry/genetics/metabolismPublished by: -
2Articles: DFG German National LicensesStaff View
ISSN: 1471-4159Source: Blackwell Publishing Journal Backfiles 1879-2005Topics: MedicineNotes: Abstract: We have previously reported that the intrinsic ability of motor axons to sprout can vary considerably from nerve to nerve with an inverse correlation to axonal length. In this study we asked whether this variation might be associated with differences in one axonal component, neurofilaments, near the site of outgrowth. The phosphorylation of epitopes on the heavy subunit of neurofilaments (NF-H) was compared in normal and regenerating axons from long and short nerves in the rat. Quantitative determination of phosphorylation states on NF-H epitopes was made by measuring immunoreactivity to monoclonal antibodies using an enzyme-linked immunosorbent assay system. Our results showed a much higher degree of phosphorylation of epitopes on NF-H in terminal portions of short compared to long axons. There was a significant inverse correlation between phosphorylation of NF-H epitopes and axonal length. In newly formed sprouts NF-H phosphorylation was reduced compared to normal. However, the absolute levels were related to the degree of NF-H phosphorylation in the parent axons. The ability to phosphorylate axonal proteins near the site of outgrowth may correlate with the potential for plastic changes in the axon such as sprouting.Type of Medium: Electronic ResourceURL: -
3Articles: DFG German National LicensesStaff View
ISSN: 1432-0533Keywords: Tubular aggregates ; Sarcoplasmic reticulum ; MRL mouse ; CastrationSource: Springer Online Journal Archives 1860-2000Topics: MedicineNotes: Summary Congenic mice of the MRL +/+ substrain provide an animal model for study of tubular aggregates in skeletal muscle. Tubular aggregates appear limited only to males of the MRL +/+ strain and are not found in other strains, including MRL +/−, MRL lpr/lpr, BXSB/MpJ, BALB/c, SJL/J, AJ, or C3H HEJ. This strain-specific occurrence, and the gradually increasing abundance of tubular aggregates after 6 months of age until virtually all type II myofibers are affected by 2 years, implies that both genetic and developmental factors are necessary for the occurrence of tubular aggregates. These two factors are not sufficient, however, since hormonal alteration by male castration nearly completely prevents the expression of tubular aggregates. Parallels are drawn with how expression of tubular aggregates is limited largely to males in diverse acquired and hereditary human neuromuscular disorders.Type of Medium: Electronic ResourceURL: -
4Articles: DFG German National LicensesStaff View
ISSN: 1432-1459Keywords: Key words Granuloma ; Inflammation ; Myopathy ; Sarcoid ; Myositis ; WeaknessSource: Springer Online Journal Archives 1860-2000Topics: MedicineNotes: Abstract We evaluated the clinical and myopathological features of all patients with granulomas in muscle biopsy specimens identified over a 5-year period (1992–1996) at the Washington University Medical Center. Ten patients were found to have granulomas in their muscle biopsy specimens. Of these, eight patients had myopathic changes. Seven had dysphagia as a major functional difficulty during the course of their disease. None had elevated levels of serum creatine kinase (CK). Four of the patients with myopathy had systemic sarcoidosis and relatively severe proximal weakness with functional disability. Treatment with corticosteroids was followed by marked improvement in strength and functional disability. The four other patients with myopathy had no systemic signs of sarcoidosis. Weakness was especially prominent distally in three of these patients. The two patients in this group treated with corticosteroids did not improve. The final two patients, who had granulomas in muscle but no myopathic changes, had clinical syndromes of mononeuritis multiplex and eosinophilic fasciitis (Shulman syndrome). We conclude that granulomatous myopathy, in the presence or absence of systemic sarcoidosis, is commonly associated with dysphagia (87%) and a normal serum CK. Clinical features in patients with sarcoidosis included severe proximal weakness with functional disability that often responded to corticosteroid treatment. Granulomatous myopathy without systemic sarcoidosis was associated with milder, but more predominantly distal weakness.Type of Medium: Electronic ResourceURL: -
5Articles: DFG German National LicensesStaff View
ISSN: 1573-7381Source: Springer Online Journal Archives 1860-2000Topics: MedicineNotes: Summary In this study we examined the distribution of cytoskeletal and other intrinsic axonal elements in motor nerve terminalsin vivo. Components of axons were visualized with immunocytochemical staining of frozen longitudinal sections of muscle. Using these methods we compared the distribution of neurofilaments, tubulin, MAP2, actin and synaptic elements in distal regions of axons at and near neuromuscular junctions in rat muscles. Our results show that three discrete regions can be defined based on the anatomy and intrinsic components of distal axons. The preterminal axon, extending from its exit from the intramuscular nerve toward the neuromuscular junction, has a cytoskeletal composition similar to the more proximal axon with abundant staining of neurofilaments, tubulin, MAP2 and actin. The terminal arborization, a branched region of the axon extending through the endplate region, contains neurofilaments but little tubulin, actin, MAP2 or synaptic elements. Finally, the synaptic zone, demonstrated with antibodies to the synaptic vesicle protein synaptophysin, contains few cytoskeletal elements. We conclude that there is considerable regional heterogeneity in the composition of distal motor axons. The distribution of neurofilaments, other cytoskeletal elements and synaptic vesicle proteins varies among different discrete zones of terminal motor axons.Type of Medium: Electronic ResourceURL: -
6Articles: DFG German National LicensesStaff View
ISSN: 1573-7381Source: Springer Online Journal Archives 1860-2000Topics: MedicineNotes: Summary We have studied the changes in the distribution of three intrinsic axonal components during the growth and maturation of sproutsin vivo. Neurofilaments, tubulin and synaptophysin, a synaptic vesicle protein, were visualized in motor axons and their sprouts using immunocytochemical staining of frozen longitudinal sections of muscle. We examined changes in these elements in sprouts regenerating after axonal crush injury and in those evoked from intact axons by denervation changes in muscle. Our results show that intrinsic axonal components move into newly formed motor axon sprouts in different temporal patterns. Based on the patterns of reorganization of staining of intrinsic axonal components, two types of outgrowth can be distinguished. One type, synaptic elaboration, is manifest by short, broad axonal processes that produce enlargement of the synaptic zone (synaptophysin staining) with little change in the distribution of intrinsic cytoskeletal elements. A second type of outgrowth, axonal elongation, occurs during axonal regeneration and ultraterminal sprouting and is longitudinal in form. In these sprouts there is a sequential appearance of neurofilament and then, several days later, tubulin immunostaining. Synaptophysin only accumulates in these sprouts after two weeks at points of synaptic contact with a muscle fibre.Type of Medium: Electronic ResourceURL: