PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “ALS”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 37 records · Page 2Linked to original sources

Expression of hepatocyte growth factor and c-Met in the anterior horn cells of the spinal cord in the patients with amyotrophic lateral sclerosis (ALS): immunohistochemical studies on sporadic ALS and familial ALS with superoxide dismutase 1 gene mutation.

To clarify the trophic mechanism of residual anterior horn cells affected by sporadic amyotrophic lateral sclerosis (SALS) and familial ALS (FALS) with superoxide dismutase 1 (SOD1) mutations, we investigated the immunohistochemical expression of hepatocyte growth factor (HGF), a novel neurotrophic factor, and its receptor, c-Met. In normal subjects, immunoreactivity to both anti-HGF and anti-c-Met antibodies was observed in almost all anterior horn cells, whereas no significant immunoreactivity was observed in astrocytes and oligodendrocytes. Histologically, the number of spinal anterior horn cells in ALS patients decreased along with disease progression. Immunohistochemically, the number of neurons negative for HGF and c-Met increased with ALS disease progression. However, throughout the course of the disease, certain residual anterior horn cells co-expressed both HGF and c-Met with the same, or even stronger intensity in comparison with those of normal subjects, irrespective of the reduction in the number of immunopositive cells. Western blot analysis revealed that c-Met was induced in the spinal cord of a patient with SALS after a clinical course of 2.5 years, whereas the level decreased in a SALS patient after a clinical course of 11 years 5 months. These results suggest that the autocrine and/or paracrine trophic support of the HGF-c-Met system contributes to the attenuation of the degeneration of residual anterior horn cells in ALS, while disruption of the neuronal HGF-c-Met system at an advanced disease stage accelerates cellular degeneration and/or the process of cell death. In SOD1-mutated FALS patients, Lewy body-like hyaline inclusions (LBHIs) in some residual anterior horn cells exhibited co-aggregation of both HGF and c-Met, although the cytoplasmic staining intensity for HGF and c-Met in the LBHI-bearing neurons was either weak or negative. Such sequestration of HGF and c-Met in LBHIs may suggest partial disruption of the HGF-c-Met system, thereby contributing to the acceleration of neuronal degeneration in FALS patients.

Adult↗

Helicobacter nemestrinae ATCC 49396T is a strain of Helicobacter pylori (Marshall et al. 1985) Goodwin et al. 1989, and Helicobacter nemestrinae Bronsdon et al. 1991 is therefore a junior heterotypic synonym of Helicobacter pylori.

Helicobacter nemestrinae Bronsdon et al. 1991, a gastric helicobacter species isolated from a pigtailed macaque, is thought to be the species most closely related to the important human pathogen Helicobacter pylori. The only available strain of this taxon is the type strain, ATCC 49396T. We sequenced seven housekeeping genes and two flagellin genes for H. nemestrinae ATCC 49396T. If ATCC 49396T were a separate species, these sequences should have been distinct from those of H. pylori. Instead, all sequences clustered together with sequences obtained previously for 20 or more H. pylori isolates from diverse geographical locations. The 16S rDNA sequence differed from that reported previously for this strain by 38 nucleotides and was most similar to that of H. pylori 85D08 (accession no. U00769), which was isolated from a rhesus macaque. It differed by less than 1% from 16S rDNA sequences of numerous other H. pylori strains, including the type strain, NCTC 11637T (= ATCC 43504T). These data indicate that the strain currently distributed as H. nemestrinae ATCC 49396T is really a strain of H. pylori and that H. nemestrinae Bronsdon et al. 1991 is a junior heterotypic synonym of Helicobacter pylori (Marshall et al. 1985) Goodwin et al. 1989.

Animals↗

Structure of the orthorhombic form of Mn(2)Al(7), Fe(2)Al(7), and (Mn(0.7)Fe(0.3))(2)Al(7) that by twinning produces grains with decagonal point-group symmetry.

Analysis of electron diffraction photographs of grains of Mn(2)Al(7), Fe(2)Al(7), and (Mn(0.7)Fe(0.3))(2)Al(7) leads to the conclusion that they are 5-fold twins of a 1664-atom orthorhombic crystal with a = 32.86 A, b = 31.23 A, and c = 24.80 A and with 16 icosahedral clusters of 104 atoms in positions shifted by small amounts from those of the cubic beta-tungsten structure.

Journal Article↗

Genomic evidence that Shouchella miscanthi (Shin et al. 2020) Joshi et al. 2022 is a later heterotypic synonym of Shouchella hunanensis (Patel and Gupta 2020) Joshi et al. 2022.

Shouchella hunanensis DSM 23008T and Shouchella miscanthi AK13T were originally described from forest soil and the rhizosphere of Miscanthus sacchariflorus, respectively. The two strains share closely similar phenotypic properties, and their 16S rRNA gene sequences show 99.5% similarity. Phylogenetic analysis of all 16S rRNA gene copies revealed copy-dependent placements, whereas the phylogenomic analysis placed the two type strains and three additional genomes in a strongly supported, short-branched cluster. Eight 16S rRNA gene copies were identified in each genome, with detectable intragenomic heterogeneity, particularly in AK13ᵀ. Pairwise average nt identity among the two type-strain genomes and three additional publicly available genomes ranged from 99.0% to 99.3%, clearly above the accepted species boundary. The digital DNA-DNA hybridization value between strains S. hunanensis DSM 23008T and S. miscanthi AK13T was 92.5%. On the basis of the combined phylogenetic, genomic and phenotypic evidence, S. miscanthi (Shin et al. 2020) Joshi et al. 2022 is proposed as a later heterotypic synonym of S. hunanensis (Patel and Gupta 2020) Joshi et al. 2022.

Phylogeny↗

Comment on papers by Chong et al., Nishio et al., and Suri et al. on diabetes reversal in NOD mice.

Chong et al., Nishio et al., and Suri et al. (Reports, 24 March 2006, pp. 1774, 1775, and 1778) confirmed that treating nonobese diabetic (NOD) mice with an immune adjuvant and semisyngenic spleen cells can reverse the disease but found that spleen cells did not contribute to the observed recovery of pancreatic islets. We show that islet regeneration predominately originates from endogenous cells but that introduced spleen cells can also contribute to islet recovery.

Animals↗

Form of Al changes with Al concentration in leaves of buckwheat.

Buckwheat (Fagopyrum esculentum Moench. cv. Jianxi) is known as an Al-accumulating plant. The process leading to the accumulation of Al in the leaves was investigated, focusing on the chemical form of Al using 27Al-nuclear magnetic resonance. Leaves with different Al concentrations were prepared by growing buckwheat on a very acidic soil (Andosol) amended with or without CaCO3 (1 or 3 g x kg-1 soil). When the Al concentration of the leaves was lower, only one major signal was observed at a chemical shift of 16.1 ppm, which was assigned to an Al-oxalate complex at a 1:3 ratio. However, when the Al concentration of the leaves increased to a high level (e.g. 12 g Al kg-1), an additional signal at a chemical shift of 11.2 ppm was observed. This signal was assigned to an Al-citrate complex at a 1:1 ratio. In the leaf with a high Al concentration, both Al-oxalate (1:3) and Al-citrate (1:1) were detected in marginal and middle parts, while only Al-oxalate was detected in the basal part. The oxalate concentration did not differ very much between leaves with low and high Al concentrations at the same position, while citrate concentration significantly increased with increasing Al concentration when the oxalate/Al ratio became lower than 3.0. As the Al-citrate complex has been demonstrated to be the form of transport in the xylem, the results suggest that when internal oxalate is enough to form a complex with Al at a 3:1 ratio in the leaves with a low Al concentration, Al-citrate converts to Al-oxalate. However, this conversion does not occur in the leaves with a very high Al concentration, resulting in the coexistence of both Al-oxalate and Al-citrate complexes.

Adaptation, Physiological↗

Isolation of the cDNA encoding the acid labile subunit (ALS) of the 150 kDa IGF-binding protein complex in cattle and ALS regulation during the transition from pregnancy to lactation.

During the transition from pregnancy to lactation, dairy cows experience a 70% reduction in plasma IGF-I. This reduction has been attributed to decreased hepatic IGF-I production. IGF-I circulates predominantly in multi-protein complexes consisting of one molecule each of IGF-I, IGF binding protein-3 and the acid labile subunit (ALS). Recent studies in the mouse have shown that absence of ALS results in accelerated turnover and severely depressed concentration of plasma IGF-I. These observations suggest that reduced plasma ALS could be a second factor contributing to the fall of plasma IGF-I in peri-parturient cows. This possibility has not been studied due to the lack of bovine ALS reagents. To address this, we isolated the bovine ALS cDNA and used its sequence to develop a ribonuclease protection assay (RPA) and a bovine ALS antiserum. Using the RPA, ALS mRNA abundance was approximately fivefold higher in liver than in lung, small intestine, adipose tissue, kidney and heart, but was absent in muscle and brain. The antiserum detected the highest ALS levels in plasma followed by ovarian follicular fluid, lymph and colostrum. A portion of colostrum and follicular fluid ALS appears to be synthesized locally as ALS mRNA was found in mammary epithelial cells and ovarian follicular cells. Finally, we measured plasma ALS in dairy cows during the peri-parturient period (days -35 and +56 relative to parturition on day 0). Plasma ALS dropped by 50% between late pregnancy and the first day of lactation and returned to prepartum levels by day +56. To determine whether this reflected a change in hepatic expression, ALS mRNA was measured in liver biopsies collected on days -35, +3 and +56. ALS mRNA expression was significantly lower on day +3 than on day -35, but recovered completely by day +56. Finally, we examined the ability of GH to increase plasma ALS abundance at selected times before and after parturition (weeks -5, -2, +1 and +5). GH increased plasma ALS at weeks -5, -2 and +5, but not at week +1. Identical effects of GH were seen when the response considered was plasma IGF-I. We conclude that the decline in plasma ALS after parturition is a consequence of hepatic GH resistance and contributes to the associated reduction of plasma IGF-I.

Amino Acid Sequence↗

The genus name Ensifer Casida 1982 takes priority over Sinorhizobium Chen et al. 1988, and Sinorhizobium morelense Wang et al. 2002 is a later synonym of Ensifer adhaerens Casida 1982. Is the combination "Sinorhizobium adhaerens" (Casida 1982) Willems et al. 2003 legitimate? Request for an Opinion.

The synonymy of the genera Ensifer and Sinorhizobium was recently reported, but it was proposed that the later-named genus, Sinorhizobium, take priority in nomenclature. There is no justification in the International Code of Nomenclature of Bacteria (Prokaryotes) for this step; Ensifer is the correct name of the genus, with Ensifer adhaerens as the type species. Species previously allocated to Sinorhizobium are here proposed as the new combinations Ensifer arboris, Ensifer fredii, Ensifer kostiensis, Ensifer kummerowiae, Ensifer medicae, Ensifer meliloti, Ensifer saheli, Ensifer terangae and Ensifer xinjiangensis. Sinorhizobium morelense was proposed in 2002 [Wang, E. T., Tan, Z. Y., Willems, A., Fernández-López, M., Reinhold-Hurek, B. & Martínez-Romero, E., Int J Syst Evol Microbiol 52, 1687-1693, 2002], but a consideration of all published data indicate that it is a nitrogen-fixing genomovar and later heterotypic synonym of Ensifer adhaerens. A Request for an Opinion is made as to whether or not the combination "Sinorhizobium adhaerens" (Casida 1982) Willems et al. 2003 is legitimate.

Phylogeny↗

Neuropathology of amyotrophic lateral sclerosis with extra-motor system degeneration: characteristics and differences in the molecular pathology between ALS with dementia and Guamanian ALS.

Amyotrophic lateral sclerosis (ALS) is classified into distinct subtypes mainly based on clinicopathological features, in addition to epidemiologic and genetic backgrounds. In addition to sporadic ALS with classical pathology, characteristics in the clinical features, in the histological findings and their topographical distribution, and in the molecular pathology, especially the intracytoplasmic neuronal inclusions, enable us to identify the following subtypes: ALS with dementia (ALS-D), ALS in the Western Pacific, ALS with multi-system degeneration, familial ALS, and superoxide dismutase 1-linked ALS. These subtypes not infrequently exhibit various types of extra-motor system degeneration, and even multi-system pathology. Some of the subtypes (for instance ALS-D or familial ALS) can be deduced, to a certain extent, from characteristic neuronal inclusions such as ALS-type ubiquitinated inclusions, Lewy body-like hyaline inclusions (LBHIs), or ubiquitinated intracytoplasmic neuronal inclusions as typically seen in the dentate fascia. The purpose of this article is to clarify the molecular pathogenesis of the cerebral cortex in ALS-D and Guamanian ALS and parkinsonism-dementia complex (PDC), in order to elucidate the relationship and distinction between these two subtypes. As indicated previously, investigations on ubiquitin-immunoreactivity in the hippocampus further support the view that the pathology of G-ALS/PDC may be that of a tau-related tangle disorder, whilst ALS-D has a feature of the motor neuron disease type-frontotemporal dementia.

Amyotrophic Lateral Sclerosis↗

[Clinical classification of ALS patients based on the total course of ALS--proposal of respiratory motor paralysis-preceded type and its significance].

Since the motor paralysis occurs in all motor systems in ALS, the clinicopathological findings and care of the patients living beyond the development of respiratory failure must be considered. We have divided the efferent motor systems into four groups, i.e., limb, bulbar, respiratory, and external ocular. In this study, 37 ALS patients were followed for more than two years with respirator, and the onset of motor paralysis in all four systems was observed in each patient. First, we classified the patients into three groups according to the number of paralyzed motor systems observed within six months from the onset of the first motor paralysis, and we discuss the patient's prognosis after the first motor paralysis. Twenty-one patients with paralysis of the respiratory motor system alone were categorized as respiratory paralysis-preceded type ALS (R-type ALS), four patients with paralysis of the bulbar motor system alone as bulbar paralysis-preceded type ALS (B-type ALS), and 12 patients with simultaneous paralysis of two or more motor systems as combined motor system paralysis type ALS (C-type ALS). The results showed that the prognosis of R-type ALS is slow progression and that of C-type ALS, rapid progression. Next, we investigated the time of occurrence of the first motor paralysis of the four motor systems in 37 patients, and we discuss the most vulnerable motor functions in ALS, i.e., the initial clinical presentations in ALS. The most frequent first motor paralysis was of the respiratory motor system, and the initial clinical manifestations in ALS were impairment of fine discriminating voluntary respiratory and limb motor functions. These findings could be explained by the rule of ALS involvements, according to which onto-genetically newer motor functions are lost earlier than the older ones. Based on the findings in this study, we concluded that ALS initially involves the ontogenetically new motor functions in "the first system" proposed by G. Holstege, whose anatomical structure is composed of pre-motoneurons and motoneurons in the brain stem and spinal cord.

Adolescent↗

Aluminium rhizotoxicity in maize grown in solutions with Al3+ or Al(OH)-4 as predominant solution Al species.

The rhizotoxicity of aluminium at low-pH with Al(3+) and at high pH with Al(OH)-(4) as the main Al species was studied. Aluminium reduced root growth to similar levels at pH 8.0 and pH 4.3, although the mononuclear Al concentration at pH 8.0 was three times lower than at pH 4.3. Al contents of root apices were much higher at pH 8 than at pH 4.3. Callose was induced only marginally at pH 8 and the formation was confined to the epidermis, whereas it proceeded through the cortex with time at pH 4.3. Well-documented genotypical differences in callose formation and Al accumulation could not be found at pH 8. The largest fraction of the root-tip Al was recovered in the cell-wall fraction independent of the solution pH. A sequential extraction of isolated cell walls suggests that most of the cell-wall Al was precipitated Al(OH)(3) at pH 8.0. This can be explained by a drastic pH reduction in the root apoplastic sap to 6.2, whereas at bulk solution pH 4.3 it rose to 5.6. Al precipitation was also confirmed by the microscopic localization of Al. At pH 8, Al could mostly be found in the epidermis, but in the apoplast of the outer cortex at pH 4.3. It is proposed here that at pH 4.3, Al(3+) inhibits root growth through binding to sensitive binding sites in the apoplast of the epidermis and the outer cortex. At pH 8, Al(OH)(3) precipitation in the epidermis causes a mechanical barrier thus impairing the root-growth control of the epidermis.

Aluminum↗

Al-Induced, 51-Kilodalton, Membrane-Bound Proteins Are Associated with Resistance to Al in a Segregating Population of Wheat.

Incorporation of 35S into protein is reduced by exposure to Al in wheat (Triticum aestivum), but the effects are genotype-specific. Exposure to 10 to 75 [mu]M Al had little effect on 35S incorporation into total protein, nuclear and mitochondrial protein, microsomal protein, and cytosolic protein in the Al-resistant cultivar PT741. In contrast, 10 [mu]M Al reduced incorporation by 21 to 38% in the Al-sensitive cultivar Katepwa, with effects becoming more pronounced (31-62%) as concentrations of Al increased. We previously reported that a pair of 51-kD membrane-bound proteins accumulated in root tips of PT741 under conditions of Al stress. We now report that the 51-kD band is labeled with 35S after 24 h of exposure to 75 [mu]M Al. The specific induction of the 51-kD band in PT741 suggested a potential role of one or both of these proteins in mediating resistance to Al. Therefore, we analyzed their expression in single plants from an F2 population arising from a cross between the PT741 and Katepwa cultivars. Accumulation of 1,3-[beta]-glucans (callose) in root tips after 24 h of exposure to 100 [mu]M Al indicated that this population segregated for Al resistance in about a 3:1 ratio. A close correlation between resistance to Al (low callose content of root tips) and accumulation of the 51-kD band was observed, indicating that at least one of these proteins cosegregates with the Al-resistance phenotype. As a first step in identifying a possible function, we have demonstrated that the 51-kD band is most clearly associated with the tonoplast. Whereas Al has been reported to stimulate the activity of the tonoplast H+-ATPase and H+-PPase, antibodies raised against these proteins did not cross-react with the 51-kD band. Efforts are now under way to purify this protein from tonoplast-enriched fractions.

Journal Article↗

Differential effects of aluminium on osmotic potential and sugar accumulation in the root cells of Al-resistant and Al-sensitive wheat.

The changes in osmotic potential and the concentration of osmotic solutes in the cell sap of the root tips exposed to Al were examined in two cultivars of wheat (Triticum aestivum) differing in Al resistance. Root elongation was less influenced by an 8-h exposure to 20 microM or 50 microM Al in Al-resistant cv. Atlas 66 than in Al-sensitive cv. Scout 66. After Al treatment the osmotic potential of the root cells was decreased in Atlas 66 but increased in Scout 66 indicating that the Al treatment osmotically stimulated the driving force for water uptake in Atlas 66 but suppressed it in Scout 66. Al increased the concentration of soluble sugars, the major osmotic solute in the root cells in Atlas 66, but decreased it in Scout 66. Al at both low (5 microM) and high (50 microM) concentrations, also increased the concentration of soluble sugars in the Al-resistant genotype ET8 but a high Al concentration decreased it in Al-sensitive genotype ES8. Enzymatic analyses and thin-layer chromatography revealed that soluble sugars in the root cells of both Atlas 66 and Scout 66 mainly consisted of monosaccharides such as glucose, fructose and a small amount of sucrose. These results suggest that the accumulation of soluble sugars in Al-resistant wheat Atlas 66 keeps the osmotic potential in the root cells low and thus, enables the root cells to take up water and to elongate against the pressure produced by cell wall rigidification under Al stress.

Journal Article↗

Multidisciplinary ALS care improves quality of life in patients with ALS.

OBJECTIVE: To examine the effect of multidisciplinary ALS care on the quality-of-life (QoL) in patients with ALS and their caregivers. METHODS: In a cross-sectional study, 208 patients with ALS and their caregivers were interviewed. QoL was assessed using the 36-item Short Form Health Survey (SF-36) and two visual analogue scales (VAS). Criteria for multidisciplinary ALS care were: an ALS team headed by a consultant in rehabilitation medicine and consisting of at least a physical therapist, occupational therapist, speech pathologist, dietician and a social worker; use of the Dutch ALS consensus guidelines for ALS care; and at least six incident ALS patients per year. RESULTS: Clinical characteristics and functional loss of the 133 patients receiving multidisciplinary ALS care and the 75 patients receiving general ALS care were similar. The percentage of patients with adequate aids and appliances was higher in those with multidisciplinary ALS care (93.1 vs 81.3%, p = 0.008), whereas the number of visits to professional caregivers was similar in both groups. Patients in the multidisciplinary ALS care group had a better mental QoL on the SF-36 Mental Summary Score than those in the general care group (p = 0.01). The difference in QoL was most pronounced in the domains of Social Functioning and Mental Health, and was independent of the presence of aids and appliances. No significant differences were found in the SF-36 Physical Summary Score, VAS, or in QoL of caregivers of patients with ALS. CONCLUSION: High standard of care improves mental quality-of-life in patients with ALS.

Aged↗

The influence of Al(III) supersaturation and NaOH concentration on the rate of crystallization of Al(OH)3 precursor particles from sodium aluminate solutions.

The growth kinetics of colloidal Al(III)-containing particles (diameter<1000 nm), nucleated in optically clear, supersaturated sodium aluminate solutions as a precursor to Al(OH)(3) crystals, has been studied using dynamic light scattering. Two series of solutions were examined at 22 degrees C to determine the influence of Al(III) supersaturation and NaOH concentration on the initial particle growth behavior. One solution series consisted of solutions with constant Al(III) absolute supersaturation (DeltaC) of 1.48 M and [NaOH] range 1.83-4.00 M ([NaOH]/[Al(III)]=1.13-2.15) and Al(III) relative supersaturation (sigma)=3.86-10.36. The other solution series had a constant sigma of 7.55 and [NaOH] range of 1.50-4.27 M ([NaOH]/[Al(III)]=1.18-1.54) and DeltaC=0.86-3.19. The correlation between the initial particle growth rates and supersaturation (DeltaC or sigma) revealed marked anomalies over the entire supersaturation range studied. The growth rate remained substantially constant in the DeltaC range 0.86-2.55 M (for the constant sigma solution series), before increasing sharply upon a further increase of DeltaC beyond 2.55 M. The variation of the growth rate with sigma in the range 3.86-9.00 (for the constant DeltaC solution series) was remarkably weak, contrary to expectation. At higher sigma (>9.00), however, a marked increase in growth rate with increasing sigma was displayed. At constant DeltaC or sigma, the growth rate showed a strong variation with NaOH concentration, indicating that Na(+) and OH(-) species play a pivotal role in the Al(OH) precursor particles (nuclei) growth process. Furthermore, the kinetics of growth displayed by these nanosized particles are an order of magnitude slower than those observed for macroscopic gibbsite (gamma-Al(OH)(3)) crystals at similar supersaturations and temperature. The difference may be rationalized in terms of particle size and Al(OH)(3) dimorphic phase dependent solubility effects. An empirically adequate growth kinetics modeling was achieved when the growth rates were correlated with the Al(III) supersaturation (DeltaC or sigma) and the excess (free) NaOH concentration, rather than the former alone, as is commonly the case. A critical [NaOH]/[Al(III)] molar ratio of 1.27-1.35, below which the particle growth rate increased markedly and above which the rate was significantly reduced, was observed. This behavior is believed to be linked to solution speciation change that occurs at certain Al(III) and NaOH compositions.

Journal Article↗

Al Inhibits Both Shoot Development and Root Growth in als3, an Al-Sensitive Arabidopsis Mutant.

In als3, an Al-sensitive Arabidopsis mutant, shoot development and root growth are sensitive to Al. Mutant als3 seedlings grown in an Al-containing medium exhibit severely inhibited leaf expansion and root growth. In the presence of Al, unexpanded leaves accumulate callose, an indicator of Al damage in roots. The possibility that the inhibition of shoot development in als3 is due to the hyperaccumulation of Al in this tissue was examined. However, it was found that the levels of Al that accumulated in shoots of als3 are not different from the wild type. The inhibition of shoot development in als3 is not a consequence of nonspecific damage to roots, because other metals (e.g. LaCl3 or CuSO4) that strongly inhibit root growth did not block shoot development in als3 seedlings. Al did not block leaf development in excised als3 shoots grown in an Al-containing medium, demonstrating that the Al-induced damage in als3 shoots was dependent on the presence of roots. This suggests that Al inhibition of als3 shoot development may be a delocalized response to Al-induced stresses in roots following Al exposure.

Journal Article↗