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At least 73 records · Page 4Linked to original sources
PubMed1987

Effects of two new aldose reductase inhibitors, AL-1567 and AL-1576, in diabetic rats.

Two new potent aldose reductase inhibitors, AL-1567 (DL-spiro(2-fluoro-9H-fluoren-9,4'-imidazolidine)-2',5'-dione) and AL-1576 (spiro-(2,7-difluoro-9H-fluoren-9,4'-imidazolidine)2',5'-dione), have been characterized with respect to in vitro activity toward rat lens and human placental aldose reductase and in vivo activity in uncontrolled, severely diabetic rats dosed acutely with the compounds. The IC50 values for inhibition of rat lens aldose reductase are 2.7 X 10(-8) mol/L for AL-1567 and 8.5 X 10(-9) mol/L for AL-1576; very similar IC50 values were measured for each compound with the human placental enzyme. When the compounds were administered orally once per day to 3-week diabetic rats for a period of eight days, the ED50 values for normalization of lens sorbitol levels were 0.60 mg/kg for AL-1567 and 0.05 mg/kg for AL-1576, and for normalization of sciatic nerve sorbitol levels; 0.22 mg/kg for AL-1567 and 0.04 mg/kg for AL-1576. Compared with published data on other aldose reductase inhibitors evaluated in very similar diabetic rat models, both compounds have unusually high activity in lens, and AL-1576 appears to be the most active such compound in both lens and sciatic nerve reported thus far. The evidence linking increased sorbitol pathway activity to diabetic complications, such as cataract and neuropathy in animal models, suggests that aldose reductase inhibitors will be useful therapeutic agents in human diabetics.

Aldehyde Reductase↗
PubMed2005

Al(3+) interaction sites of calmodulin and the Al(3+) effect on target binding of calmodulin.

The interaction between calmodulin (CaM) and Al(3+) was studied by spectroscopic methods. Heteronuclear two-dimensional NMR data indicated that peaks related to the both lobes and middle of the central helix of CaM are largely affected by Al(3+). But chemical shift perturbation suggested that overall conformation of Ca(2+)-loaded CaM is not changed by Al(3+) binding. It is thought that Al(3+) interaction to the middle of the central helix is a key for the property of CaM's target recognition. If the structure and/or flexibility of the central helix are/is changed by Al(3+), target affinity to CaM must be influenced by Al(3+). Thus, we performed surface plasmon resonance experiments to observe the effect of Al(3+) on the target recognition by CaM. The data clearly indicated that target affinity to CaM is reduced by addition of Al(3+). All the results presented here support a hypothesis that Al(3+) may affect on the Ca(2+) signaling pathway in cells.

Aluminum↗
PubMed2001

Direct determination of the permanent dipole moments and structures of Al-CH(3)CN and Al-NH(3) by using a 2-m electrostatic hexapole field.

The supersonic beams of the (1-1) metal-ligand complexes of Al-CH(3)CN and Al-NH(3) were produced by a laser evaporation method. Nondestructive structure selection of the complexes and the dipole moment determination were performed by using a 2-m electrostatic hexapole field. The experimentally determined permanent dipole moments are 1.2 +/- 0.1 D for Al-CH(3)CN and 2.7 +/- 0.2 D for Al-NH(3). We find that the dipole moment of Al-NH(3) becomes larger than that of neat NH(3), while the formation of the Al-CH(3)CN complex produces a smaller dipole moment than that of neat CH(3)CN on the other hand. We performed the ab initio calculations to draw out plausible complex structures and to clarify the bonding character after formation of the complex, and we made comparisons with the computational results done by several groups. The Mulliken population analysis suggests the Al-->CH(3)CN charge flow, but on the other hand the Natural population analysis indicates very little charge flow. For the Al-NH(3) complex, the polarization effect of NH(3) and the N-->Al sigma donation would enhance the dipole moment strength. However, there still remains a controversial disagreement between the theoretical predictions and the experimental results. Further experimental determination using the hexapole method for various metal-ligand complexes and clusters could reveal the basic nature of interaction in the complex systems in general, and this method would complement theoretical calculations.

Journal Article↗
PubMed2005

Influence of the Al source and synthesis of ordered Al-SBA-15 hexagonal particles with nanostairs and terraces.

We investigated the effect of various aluminum sources on the morphology and microstructure of nanoporous Al-SBA-15. With aluminum tri-sec-butoxide (Al-TSB), very large, ordered hexagonal rods of Al-SBA-15 covered by nanostairs and terraces were synthesized. Such a morphology was not obtained with an inorganic Al source (sodium aluminate) or with organic sources seemingly similar to Al-TSB, such as aluminum tri-tert-butoxide, aluminum tri-n-butoxide, or aluminum isopropoxide. The results obtained from X-ray diffraction, scanning electron microscopy/transmission electron microscopy, N(2) adsorption/desorption, and high-performance (129)Xe NMR suggest that preparing an organic Al-Si precursor by premixing liquid organic Al and Si sources (Al-TSB and tetraethyl orthosilicate) is crucial in obtaining highly ordered mesoporous Al-SBA-15 materials with a well-defined morphology.

Journal Article↗
PubMed1999

[3H]AL-5848 ([3H]9beta-(+)-Fluprostenol). Carboxylic acid of travoprost (AL-6221), a novel FP prostaglandin to study the pharmacology and autoradiographic localization of the FP receptor.

AL-5848 (5Z,13E)-(9 S,11R,15S)-9,11,15-trihydroxy-5,13-prostadienoic acid) is the carboxylic acid of travoprost (AL-6221), a single (+)-isomer of (+/-)-fluprostenol, an FP-class prostaglandin agonist which lowers intraocular pressure. We have prepared a radioligand from this selective prostaglandin and demonstrated its utility for studying the pharmacology and autoradiographic location of the FP-receptor. Specific [3H]AL-5848 binding (84% of total) was linearly related to bovine corpus luteum tissue concentration and reached equilibrium within 275 min at 23 degrees C. Scatchard analysis of saturation isotherms indicated interaction of [3H]AL-5848 with a single class of high-affinity (dissociation constant, Kd, = 33.8+/-2.9 nM, n = 4) and saturable (Bmax = 37.3+/-3.0 pmol (g wet weight tissue)(-1)) FP receptor-binding sites in bovine corpus luteum. Specific [3H]AL-5848 binding was potently inhibited by the FP-receptor ligands 16-phenoxyPGF2alpha (inhibition constant Ki = 17.3 nM); cloprostenol (Ki = 56.8 nM); 17-phenyl PGF2alpha (Ki = 87.0 nM); AL-5848 (Ki = 52.1 nM); PGF2alpha (Ki = 195 nM); PHXA85 (Ki = 223 nM); (n = 3-11) but very weakly by PGD2, ZK118182, BW245C, PGE2, PGI2 and U-46619. The pharmacology of specific [3H]AL-5848 binding correlated well with the pharmacology of [3H]PGF2alpha binding in the bovine corpus luteum preparation (r = 0.98, n = 14, P<0.0001) and also with functional responses in Swiss 3T3 and rat vascular smooth muscle cells (A7r5) (r = 0.96) expressing FP receptors. Autoradiographic studies revealed high levels of specific FP-receptor binding with [3H]AL-5848 on granulosa cells in the bovine corpus luteum sections, and on longitudinal ciliary muscle, the ciliary process, the iris sphincter and the retina in eye sections from man. These studies show [3H]AL-5848 to be a high-affinity agonist radioligand capable of selectively labelling the FP prostaglandin receptor.

3T3 Cells↗
PubMed1994

Fragmentation of the Golgi apparatus of motor neurons in amyotrophic lateral sclerosis (ALS). Clinical studies in ALS of Guam and experimental studies in deafferented neurons and in beta,beta'-iminodipropionitrile axonopathy.

Previous morphological immunoenzymatic studies with organelle-specific antibodies have disclosed an apparent fragmentation of the Golgi apparatus in large numbers of motor neurons in 12 cases of sporadic, non-Guamanian amyotrophic lateral sclerosis (ALS) in three cases of other types of motor neuron disease and in one case of a mitochondrial myopathy with cytochrome c oxidase deficiency. Motor neurons with fragmented Golgi apparatus were moderately atrophic; in these cells, discrete immunostained elements of the organelle were twice as many as in normal neurons, and the size of each Golgi element and the percentage of the cytoplasmic area occupied by the Golgi apparatus were reduced (Am J Pathol 1992, 140: 731-737). In this report we have confirmed the fragmentation of the organelle of motor neurons in the spinal cord in six sporadic cases of Guamanian ALS. In four of the six cases the clinical course was 1 to 2 years. The percentages of motor neurons with fragmented Golgi apparatus varied from 38 to 92. Motor neurons from three additional cases of Guamanian ALS of clinical duration from 5 to 7 years did not show fragmentation of the Golgi apparatus. In two cases of Guamanian ALS and in one non-Guamanian ALS, all neurons with ubiquitin-positive skein-like or granular inclusions believed to be pathognomonic for ALS had fragmented Golgi apparatus. To examine whether the fragmentation of the Golgi apparatus results from reactions to either neuronal deafferentation or to lesions of proximal axons, we conducted two experimental studies. In the first study, we examined in cats the Golgi apparatus of deafferented neurons of the dorsal lateral geniculate nucleus. In the second study, we examined the Golgi apparatus of motor neurons in the spinal cord of rats with proximal axonopathy induced by beta,beta'-iminodipropionitrile. In these two experiments, the neuronal Golgi apparatus studied by immunoenzymatic techniques and morphometry, was not fragmented. Taken together, the results of these studies strongly suggest that the fragmentation of the Golgi apparatus of motor neurons in ALS represents an important and perhaps early change of the organelle that may be involved in the pathogenesis of ALS. The fragmentation of the Golgi apparatus of motor neurons is a fairly specific and easily recognizable marker of ALS and may be used together with other criteria for comparisons between the human disease and proposed animal models of the disorder.

Adult↗
PubMedDate not supplied

Further studies on the erythrocyte uptake of lead in vitro in amyotrophic lateral sclerosis (ALS) patients and controls. Abnormal erythrocyte fragility in ALS.

Following our earlier observations on increased plasma concentrations of lead in amyotrophic lateral sclerosis (ALS), the erythrocyte uptake of lead from plasma has been studied in vitro. Whole-blood from ALS patients and controls was incubated after the addition of lead (0.6 mumol/l whole-blood) and plasma lead concentrations were repeatedly determined. Incubation was continued until haemolysis developed. Fairly stable plasma lead concentrations were established at, on the average, 0.5-0.6 mumol/l after 10-30 min and persisted throughout the incubation with no significant difference between ALS- and control samples. Unexpectedly, it was also observed that haemolysis occurred significantly earlier in the ALS- than in the control samples. Plateau levels in plasma lead concentration of the same order as in the present experiments have been observed both in ALS- and control samples in previous experiments with the same technique, where the lead dose added was twice as high, and these plateau levels were about 10 times higher than those observed in vivo in ALS patients and controls. It is therefore suggested that the final plasma lead concentrations in vivo is established by factors other than the erythrocyte uptake and it is improbable that the differences between ALS patients and controls in plasma lead concentration are associated with differences in the degree of lead uptake by the red cells. The increased plasma lead concentrations in ALS patients may instead be caused by increased fragility of the erythrocytes, as manifested by the earlier occurrence of haemolysis in the present experiments. The observation of increased red cell fragility is, however, also of interest as a possible manifestation of a generalized membrane defect.

Aged↗
PubMed2000

Risk factors in the early diagnosis of ALS: North American epidemiological studies. ALS CARE Study Group.

Patient-specific (endogenous) and population-specific (exogenous) risk factor analysis is identifying novel physical and chemical exposures which might be time-linked to the development of amyotrophic lateral sclerosis (ALS) and other motor neuron diseases. Electric injury in a number of case-control studies as well as prolonged exposure at work and home to agricultural chemicals in pesticides and herbicides have been identified as significant risk factors. Heavy exercise, trauma with or without bone fractures and heavy metal exposure at work have not been confirmed as risk factors. Surprisingly, occupation as a pilot or navigator has recently been identified as a potential risk factor, which will need to be confirmed. The introduction of international patient registries in North America (ALS CARE) and in Europe (ALS HPS) will facilitate future studies on the prognosis of ALS, adherence to standards of practice, quality of life and patient outcome studies. An initial survey of the ALS Patient Care Database in January 1999, when nearly 1800 patients had been entered across North America, indicated the median time from ALS onset to diagnosis is 14 months when no second opinion is requested, 12 months if the patient requests a second opinion and 10 months when the neurologist requests an additional opinion. No significant difference was found in the median time to diagnose sporadic ALS patients compared with familial ALS patients.

Case-Control Studies↗
PubMed1997

Possible Involvement of Al-Induced Electrical Signals in Al Tolerance in Wheat.

The relationship between Al-induced depolarization of root-cell transmembrane electrical potentials (Em) and Al tolerance in wheat (Triticum aestivum L.) was investigated. Al exposure induced depolarizations of Em in the Al-tolerant wheat cultivars Atlas and ET3, but not in the Al-sensitive wheat cultivars Scout and ES3. The depolarizations of Em occured in root cap cells and as far back as 10 mm from the root tip. The depolarization was specific to Al3+; no depolarization was observed when roots were exposed to the rhizotoxic trivalent cation La3+. The Al-induced depolarization occurred in the presence of anion-channel antagonists that blocked the release of malate, indicating that the depolarization is not due to the electrogenic efflux of malate2-. K+-induced depolarizations in the root cap were of the same magnitude as Al-induced depolarizations, but did not trigger malate release, indicating that Al-induced depolarization of root cap cell membrane potentials is probably linked to, but is not sufficient to trigger, malate release.

Journal Article↗
PubMed2000

The ALS patient care database: goals, design, and early results. ALS C.A.R.E. Study Group.

OBJECTIVE: The ALS Patient Care Database was created to improve the quality of care for patients with ALS by 1) providing neurologists with data to evaluate and improve their practices, 2) publishing data on temporal trends in the care of patients with ALS, and 3) developing hypotheses to be tested during formal clinical trials. BACKGROUND: Substantial variations exist in managing ALS, but there has been no North American database to measure outcomes in ALS until now. METHODS: This observational database is open to all neurologists practicing in North America, who are encouraged to enroll both incident and prevalent ALS patients. Longitudinal data are collected at intervals of 3 to 6 months by using standard data collection instruments. Forms are submitted to a central data coordinating center, which mails quarterly reports to participating neurologists. RESULTS: Beginning in September 1996 through November 30, 1998, 1,857 patients were enrolled at 83 clinical sites. On enrollment, patients had a mean age of 58.6 years +/-12.9 (SD) years (range, 20.1 to 95.1 years), 92% were white, and 61% were men. The mean interval between onset of symptoms and diagnosis was 1.2+/-1.6 years (range, 0 to 31.9 years). Riluzole was the most frequently used disease-specific therapy (48%). Physical therapy was the most common nonpharmacologic intervention (45%). The primary caregiver was generally the spouse (77%). Advance directives were in place at the time of death for 70% of 213 enrolled patients who were reported to have died. CONCLUSIONS: The ALS Patient Care Database appears to provide valuable data on physician practices and patient-focused outcomes in ALS.

Activities of Daily Living↗
PubMed1991

[Environmental factors in western Pacific foci of ALS and a possible pathogenetic role of aluminum (Al) in motor neuron degeneration].

Recent decline in incidence rates of ALS in Guam and the Kii Peninsula of Japan strongly implicates environmental factors rather than inheritance in its causation. Environmental studies in Western Pacific foci showed identical mineral compositions in the soils and drinking water, i.e., extremely low calcium (Ca) and magnesium (Mg) and high aluminum (Al) and manganese (Mn). Series of trace-elemental analyses of the CNS tissue of ALS patients have revealed a high contents of Al and Ca with significant positive correlations between Al and Ca and/or between Ca and Mn, suggesting the prolonged exposure to these trace environment to cause abnormal mineral metabolism detrimental to neurons. Using electron energy loss spectrometry (EELS), Al was found to accumulate within DNA-containing chromatins and rRNA-containing cellular components, i.e., nucleolus, heterochromatin, rough endoplasmic reticulum, in lumbar motor neurons of ALS. Thus, Al may preferentially bind to nucleic acids and cause a progressive inhibition of the protein synthesis of rRNA and the transcription or gene modulation of DNA, leading to neuronal degeneration.

Aluminum↗
PubMed1995

Minimal effects of two aldose reductase inhibitors, AL-1576 and AL-4114, after subacute topical-ocular dosing on xenobiotic biotransformation in rabbits.

Aldose reductase is believed to be involved in teh etiology of diabetic complications, including cataractogenesis, nephropathy, and neuropathy. AL-1576 and AL-4114, two spirohydantoin aldose reductase inhibitors, were specifically developed for prevention of diabetic cataractogenesis. This study has determined whether AL-1576 and AL-4114 are inducers of biotransformation by assaying the activities of some phase I and phase II enzymes in the liver, kidney, intestine, and five ocular tissues (cornea, lens, iris-ciliary body, retina, and choroid). The aldose reductase inhibitors were administered topically (the intended route for use in preventing cataractogenesis) in two concentrations (0.5 and 5.0%) each 3 times/day to both eyes of New Zealand white rabbits for 14 days. Lenticular aldose reductase activity was decreased by 30-75% by the aldose reductase inhibitors. Monooxygenase activity toward benzo(a)pyrene, ethoxyresorufin, and methoxycoumarin was not increased by AL-1576 or AL-4114 treatment in any tissue. Activities of 1-chloro-2,4-dinitrobenzene glutathione S-transferase, 2-naphthol sulfotransferase, and 1-naphthol UDP-glucuronosyltransferase were not significantly induced in the eight tissues. Clearly, ocular dosing with AL-4114 and AL-1576 for 14 days had little effect on hepatic, intestinal, and ocular biotransformation.

Administration, Topical↗
PubMed2001

The ALS Patient Care Database: insights into end-of-life care in ALS.

OBJECTIVE: To study clinical practices and patient outcomes near the end of life in amyotrophic lateral sclerosis (ALS). BACKGROUND: Patients, families, and healthcare providers face several dilemmas in selecting and delivering care near the end of life in ALS. Published data on clinical practices and their benefits during end-of-life care for ALS patients consist of anecdotal reports based on small case series or individual case reports. METHODS: Data were obtained from 1014 American and Canadian patients with ALS who died while participating in a large observational registry (the ALS Patient Care Database) during the past four years. Following death, a caregiver or family member provided data for each patient using a standard questionnaire. Data were principally generated through American and Canadian ALS multidisciplinary centers of excellence. RESULTS: Most patients died peacefully (90.7%) and 62.4% died in a hospice-supported environment. Advance directives were in place for 88.9% of patients and were followed in 96.8%. Among the 67 patients who exhibited distress in the dying process, symptoms included breathing difficulties (82.1%), fear/anxiety (55.2%), pain (23.9%), insomnia (14.9%), and choking (14.93%). Oxygen was given to 52.6% of patients, and pain medications were given to 74%. CONCLUSION: These data suggest that palliative care at the end of life was relatively well managed for most patients with ALS who participated in this study; nevertheless, several opportunities for improvement were identified.

Amyotrophic Lateral Sclerosis↗
PubMed2001

Current management of ALS: comparison of the ALS CARE Database and the AAN Practice Parameter. The American Academy of Neurology.

BACKGROUND: The American Academy of Neurology (AAN) ALS Practice Parameter was published in April 1999. The ALS CARE Database has been collecting data on the management of patients with ALS in North America since 1996. OBJECTIVE: To compare the management of patients with ALS in North America as recorded in the ALS CARE Database with the recommendations of the AAN ALS Practice Parameter. METHODS: Data were analyzed from 2018 patients at enrollment and from 373 of these patients who died between enrollment and May 1999. RESULTS: Eighty-two percent of the enrolled patients reported that they had been given enough information about ALS. Only 54% of patients with drooling were receiving medication for this problem. Only 41% of those who reported being depressed most of the time were receiving antidepressant medications. Only 28% of those with dyspnea and only 9.2% of those with a forced vital capacity <40% predicted were receiving noninvasive positive pressure ventilator support. Only 30% of those with moderate to severe dysphagia had a gastrostomy tube. Half of the patients who died did so at home, but only 47% of them received residential hospice services. Although 89% of patients who died were recorded as having done so peacefully, 17% were reported to have had breathing difficulties (i.e., respiratory distress), 8% anxiety, 3.3% pain, and 2.5% choking. Advance directives were in place for 90% of the patients who died, and in 97% of cases these directives were followed. CONCLUSIONS: These findings indicate that in the 3-year period prior to the publication of the AAN Practice Parameter, many but not all patients received the care that is recommended in that parameter; there were deficiencies, particularly in the key areas of gastrostomy and noninvasive positive pressure ventilation.

Amyotrophic Lateral Sclerosis↗
PubMed2006

Electron paramagnetic resonance study of the mononuclear Al species formed in the reaction of Al atoms and cyclopropylamine at 77 k in an adamantane matrix.

Ground-state Al atoms have been reacted with cyclopropylamine (CpNH2) in an adamantane matrix at 77 K. The four paramagnetic species generated in the reaction have been studied by electron paramagnetic resonance (EPR) spectroscopy and their magnetic parameters extracted from the EPR spectra. The isotropic Al hyperfine interactions (hfis) for these radicals vary from 801 to 962 MHz and are consistent with values previously reported for structurally similar Al-centered radicals. The major species formed in the reaction has a highly anisotropic a tensor indicative of an Al-centered radical with a substantial amount of the unpaired spin density residing in the Al 3p orbital. In addition, there is EPR evidence that the insertion products, CpNH(AlH) and CpAlNH2, are formed in the reaction, suggesting that Al atoms can activate the N-H and C-N bonds of amines. It has been shown that a molecule of CpNH2 coordinates to CpNH(AlH), causing the Al, N and H hfi of the N-H insertion product to decrease. The values of the nuclear hfis calculated for the insertion products, using a density functional theory (DFT) method, are comparable to the experimental values.

Adamantane↗
PubMed2003

Comparison of body mass index values proposed by Cole et al. (2000) and Must et al. (1991) for identifying obese children with weight-for-height index recommended by the World Health Organization.

OBJECTIVES: To calculate the sensitivity, specificity and agreement of body mass index (BMI) values proposed by Cole et al. (Br. Med. J. 2000; 320: 1) and Must et al. (Am. J. Clin. Nutr. 1991; 53: 839 & 54: 773) with weight-for-height index in the nutritional evaluation of children. DESIGN: Criterion standards for diagnostic tests. SETTING: : North-east and south-east Brazil. SUBJECTS: Two thousand nine hundred and twenty children studied in Life Pattern Research performed by the Brazilian Institute of Geography and Statistics in 1997. Main outcome measures are the sensitivity, specificity and agreement of BMI values proposed by Must et al. (1991) and Cole et al. (2000). RESULTS: Sensitivity of values proposed by both authors was around 90%. Specificity was almost 100% considering weight-for-height index as the gold standard. The agreement of both values with weight-for-height index, based on kappa results, was good and in pre-school children it was excellent. CONCLUSIONS: Values proposed by Cole et al. (2000) and Must et al. (1991) should be used carefully to screen obesity in childhood but can be used to "diagnose" overweight children with a very low chance of having false-positive results. Although the values proposed by both authors performed similarly, use of Cole et al.'s values should be encouraged. The latter cover children from 2 to 6 years old; their values are presented for six-month age intervals; they are based on a larger sample from six different countries; and they are related to the definition of adult obesity.

Body Height↗
PubMed2006

Differential expression of GABAA and glycine receptors in ALS-resistant vs. ALS-vulnerable motoneurons: possible implications for selective vulnerability of motoneurons.

Summary Amyotrophic lateral sclerosis (ALS) is a devastating motoneuronal degenerative disease, which is inevitably fatal in adults. ALS is characterized by an extensive loss of motoneurons in the cerebrospinal axis, except for those motoneurons that control eye movements and bladder contraction. The reason for this selectivity is not known. Systematic differences have been found in the organization of excitatory synaptic transmission in ALS-resistant vs. ALS-susceptible motor nuclei. However, although motoneurons express high levels of glycine receptors (GlyR) and GABA(A) receptors (GABA(A)R), no such studies have been carried out yet for inhibitory synaptic transmission. In this study, we compared the subunit composition, patterns of expression, density and synaptic localization of inhibitory synaptic receptors in ALS-resistant (oculomotor, trochlear and abducens) and ALS-vulnerable motoneurons (trigeminal, facial and hypoglossi). Triple immunofluorescent stainings of the major GABA(A)R subunits (alpha1, alpha2, alpha3, and alpha5), the GlyR alpha1 subunit and gephyrin, were visualized by confocal microscopy and analysed quantitatively. A strong correlation was observed between the vulnerability of motoneurons and the subunit composition of GABA(A)R, the GlyR/GABA(A)R density ratios and the incidence of synaptic vs. extrasynaptic GABA(A)R. These differences contrast strikingly with the uniform gephyrin cluster density and synaptic GlyR levels recorded in all motor nuclei examined. These results suggest that the specific patterns of inhibitory receptor organization observed might reflect functional differences that are relevant to the physiopathology of ALS.

Adult↗
PubMed1996

ALS care: a resource for measuring and improving ALS outcomes.

The Amyotrophic Lateral Sclerosis Clinical Assessment, Research, and Education Project (ALS CARE) will conduct outcomes research and develop educational programs that benefit ALS patients and neurologists. An advisory board of neurologists, who are experts in ALS, will establish the policies governing this project and control the dissemination of aggregate data on ALS practices and outcomes. As a first step toward improving the care of ALS patients, a data coordinating center has been established in the Center for Outcomes Research at the University of Massachusetts Medical Center, which will manage a North American Database of ALS Outcomes. This voluntary database is designed to (1) guide the development of educational programs to improve the care of ALS patients and (2) provide a mechanism for neurologists to evaluate the impact of their diagnostic and therapeutic decisions in a manner that is timely, confidential, and objective.

Amyotrophic Lateral Sclerosis↗