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At least 1,369 records · Page 76Linked to original sources
PubMed2004

Drosophila spastin regulates synaptic microtubule networks and is required for normal motor function.

The most common form of human autosomal dominant hereditary spastic paraplegia (AD-HSP) is caused by mutations in the SPG4 (spastin) gene, which encodes an AAA ATPase closely related in sequence to the microtubule-severing protein Katanin. Patients with AD-HSP exhibit degeneration of the distal regions of the longest axons in the spinal cord. Loss-of-function mutations in the Drosophila spastin gene produce larval neuromuscular junction (NMJ) phenotypes. NMJ synaptic boutons in spastin mutants are more numerous and more clustered than in wild-type, and transmitter release is impaired. spastin-null adult flies have severe movement defects. They do not fly or jump, they climb poorly, and they have short lifespans. spastin hypomorphs have weaker behavioral phenotypes. Overexpression of Spastin erases the muscle microtubule network. This gain-of-function phenotype is consistent with the hypothesis that Spastin has microtubule-severing activity, and implies that spastin loss-of-function mutants should have an increased number of microtubules. Surprisingly, however, we observed the opposite phenotype: in spastin-null mutants, there are fewer microtubule bundles within the NMJ, especially in its distal boutons. The Drosophila NMJ is a glutamatergic synapse that resembles excitatory synapses in the mammalian spinal cord, so the reduction of organized presynaptic microtubules that we observe in spastin mutants may be relevant to an understanding of human Spastin's role in maintenance of axon terminals in the spinal cord.

Adenosine Triphosphatases↗
PubMed1996

Effect of aging on the esophageal motor functions.

To clarify the changes of esophageal motility along with age, we performed esophageal manometry on 47 healthy volunteers, and compared the values of four groups under 49 years old (n = 11), 50 to 59 (n = 15), 60 to 69 (n = 11), and over 70 years old (n = 10). Resting lower esophageal sphincter (LES) pressure in the group over 70 years old showed the tendency of decrease, but not statistically significant. Nadir LES pressure on swallow-induced relaxation was not statistically different among 4 groups. On esophageal body testing, percentage of non-conduction sequence in the group 60 to 69 and over 70 years old was statistically higher compared with that of the group under 49 years old (p < 0.05) but spared in some elderly subjects. Percentage of simultaneous contractions was not statistically different among 4 groups. Peristaltic contraction amplitude in the group over 70 years old was significantly lower than that of the group under 49 years old both at the level of 5 cm above (p < 0.01) and 10 cm above LES (p < 0.05). We speculate that the influence of aging on esophageal motility is the reduced transmission sequence of peristalsis and contractility of esophageal body. This alteration along with age may differ from the pathological condition of scleroderma or diffuse esophageal spasm.

Adult↗
PubMed1986

[Studies on the motor function of canine gastric tube].

The need for a drainage procedure to facilitate emptying of the gastric tube remains a controversial issue. Then to elucidate whether we need a drainage procedure or not, on both gastric tube without drainage and with drainage, the author investigated the electromyographical changes of antrum and the manometric changes of pyloric portion before and after expansive stimulation, and observed the emptying of gastric tube. The results are summarized as follows: 1. In the gastric tube without drainage procedure, the prolongation of discharge intervals and the acceleration of propagation velocity were noted electromyographically, and the increase of pressure of pyloric portion was noted. When expansive stimulation was added, these findings were more enhanced. 2. In the gastric tube with pyloromyotomy, the states of electromyographical and manometrical excitation were weakened as compared with the gastric tube without drainage, and the reaction to expansive stimulation was also decreased. 3. By addition of pyloromyotomy, the emptying of gastric tube was more accelerated and the pattern of gastric emptying became nearly stabilized. The above results suggested that the gastric tube may need a drainage procedure to facilitate emptying.

Animals↗
PubMedDate not supplied

[Validation of the EK functional motor scale in the Portuguese language].

OBJECTIVE: To validate a Portuguese version of the EK scale (Egen Klassifikation), that was developed in Denmark for patients with Duchenne muscular dystrophy, and spinal muscular atrophy. METHODS: An English version of the EK scale was translated into the Portuguese language as spoken in Brazil. This scale was applied to 26 male patients (mean age = 12.7 +/- 4.0 years) with Duchenne muscular dystrophy. Patients also answered questions of a Portuguese version of the Barthel index questionnaire, and had their right and left hand grip forces measured with a dynamometer. RESULTS: The mean total EK scale score was 8.1 +/- 7.3 and the Barthel index 54.0 +/- 26.2. The mean hand grip force was 12.7 +/- 17.2 % predicted for the right hand, and 14.6 +/- 19.8 % predicted for the left hand. The EK scale scores showed highly significant statistical correlations with age (r= 0.596, p= 0.0013), right hand grip force (r= -0.556, p= 0.0032), left hand grip force (r= -0.623, p=0.0007), and Barthel index (r= -0.928, p < 0.0001). CONCLUSION: This translated Portuguese version of the EK scale is an adequate tool to be used for Brazilian Duchenne muscular dystrophy patients.

Adolescent↗
PubMed1992

Recovery of impaired motor function of the upper extremity after stroke.

Manual function score (MFS) of 174 hemiparetic stroke patients was examined every week for 8 weeks after starting the occupational therapy with programs based on MFS recovery profile, and the relation between the time since stroke onset (x) and MFS (y) was approximated by a hyperbolic function, y = A - B/x. The patients were classified into two groups, 125 cases (71.8%) with statistically significant fit of the function (fit group) and 49 without fit (non-fit). Using demographic and neurological variables of each patient, characteristics of the patients with regular recovery were analyzed. The results indicated that the gain of MFS during 8 weeks of the therapy was large in the fit group compared to the non-fit, and the regular recovery could be mostly expected in rather young patients, those with early start of the therapy, and those without ataxia, mental deterioration, and cortical lesions.

Arm↗
PubMed2003

Functional motor neurons differentiating from mouse multipotent spinal cord precursor cells in culture and after transplantation into transected sciatic nerve.

OBJECT: One of the current challenges in neurobiology is to ensure that neural precursor cells differentiate into specific neuron types, so that they can be used for transplantation purposes in patients with neuron loss. The goal of this study was to determine if spinal cord precursor cells could differentiate into motor neurons both in culture and following transplantation into a transected sciatic nerve. METHODS: In cultures with trophic factors, neurons differentiate from embryonic precursor cells and express motor neuronal markers such as choline acetyltransferase (ChAT), Islet-1, and REG2. Reverse transcription-polymerase chain reaction analysis has also demonstrated the expression of Islet-1 in differentiated cultures. A coculture preparation of neurospheres and skeletal myocytes was used to show the formation of neuromuscular connections between precursor cell-derived neurons and myocytes both immunohistochemically and electrophysiologically. Following various survival intervals, precursor cells transplanted distal to a transection of the sciatic nerve differentiated into neurons expressing the motor neuron markers ChAT and the alpha1 1.2 (class C, L-type) voltage-sensitive Ca++ channel subunit. These cells extended axons into the muscle, where they formed cholinergic terminals. CONCLUSIONS: These results demonstrate that motor neurons can differentiate from spinal cord neural precursor cells grown in culture as well as following transplantation into a transected peripheral nerve.

Animals↗