Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P01189 (beta-endorphin)
 21,003 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A new combination therapy, high-dose pyridoxal phosphate (40 to 50 mg/kg daily) and low-dose corticotropin (0.01 mg [0.4 IU]/kg daily), was tried in 28 children with infantile spasms. Monotherapy with pyridoxal phosphate provided excellent seizure control in three (11%) of the 28 subjects. Corticotropin was subsequently added to the regimen of the remaining 25 patients. At 1 month after discontinuing corticotropin, 21 (84%) of the 25 patients experienced no seizures, and 22 (88%) of the 25 showed improvement in their electroencephalographic findings. The mean interval until achievement of seizure control was 4.1 days after the initiation of corticotropin. The outcome in the 21 patients has been followed for a mean period of 34.9 months (range, 2 to 81 months). Of these 21 patients, six (29%) have had relapses of infantile spasms, and 10 (48%) have experienced normal development. Transient increases in liver enzymes occurred in 14 (50%) of the 28 patients, but none of the patients developed more serious side effects. The investigators conclude that combination therapy with high-dose pyridoxal phosphate and low-dose corticotropin is a promising new therapy.
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PMID:Combination therapy of infantile spasms with high-dose pyridoxal phosphate and low-dose corticotropin. 874 83

Eighteen children with West syndrome (5-11 months of age) were selected to receive an oral dose of pyridoxal phosphate, (20-50 mg/kg) for 14 d. Seizures disappeared in one patient. The remaining 17 patients were treated with 0.01 mg/kg synthesized corticotropin intramuscularly for 2 weeks as an additional therapy. Seizures disappeared in all 17 patients within a few days after initiation of the corticotropin. Levels of somatostatin in the cerebrospinal fluid were as follows: 61.0+/-10.7 pg/ml before therapy, 34.2+/-6.4 pg/ml during pyridoxal phosphate therapy, and 26.8+/-4.2 pg/ml after 2 weeks corticotropin therapy. Somatostatin levels in untreated patients were higher (p < 0.05) than those of age-matched controls (35.7+/-11.8 pg/ml) and decreased (p < 0.05) after pyridoxal phosphate treatment. Somatostatin is a hypothalamic tetradecapeptide with excitatory effects on neurons and pyridoxal phosphate might subclinically influence neuronal excitation.
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PMID:Cerebrospinal fluid somatostatin in West syndrome: changes in response to combined treatment with high-dose pyridoxal phosphate and low-dose corticotropin. 992 Apr 58

The case of a 5-month-old boy with tuberous sclerosis and West syndrome is reported. Tonic spasms were noted from the age of 4 months. High-dose pyridoxal phosphate could not control the seizures completely. Very short and low-dose adrenocorticotropic hormone (ACTH) therapy (i.e. 0.011 mg/kg per dose, 12 times in 20 days) controlled the seizures, while pyridoxal phosphate was on. Early tapering of ACTH was successfully done while abnormal electroencephalogram (EEG) findings remained. Although side effects such as hypertension and brain shrinkage were transiently observed, both the cognitive and seizure prognoses were excellent at the age of 3 years and 2 months. The good response to a small dosage of ACTH might be due to some responsiveness of the high-dose pyridoxal phosphate and the underlying cause of tuberous sclerosis with normal development before onset. The present case illustrates that the duration and dosage of ACTH therapy in West syndrome should be modified according to the individual's requirements.
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PMID:A case of West syndrome well controlled by very short and low-dose ACTH therapy. 1020 Dec 87

We sent questionnaires concerning the current therapy for West syndrome to 208 institutions at which pediatric care members of the Japan Epilepsy Society were working. Of these, 129 (62%) institutions responded. Vitamin B6 was the preferred first-line drug, followed by the combination of vitamin B6 and valproate or monotherapy with valproate. Corticotropin was the third choice among the drugs. The dosage of corticotropin was lower than previously reported. The treatment of West syndrome is not well established at present and further research is needed to improve the therapeutic protocol.
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PMID:Current therapy for West syndrome in Japan. 1086 90

The purpose of this study was to investigate the metabolism of Adenosine triphosphate (ATP) in skeletal muscle resistance arterioles and to determine whether this metabolism is altered during the rapid growth phase of the rat. We attempted to quantify ATP metabolism in gastrocnemius first-order arterioles from 8-, 10-, and 12-week-old rats. We measured ATP metabolism using an ATPase/GTPase assay with whole vessel segments as well as using a real-time adenosine biosensor following electric field stimulation. Our first method of measuring ATP metabolism allowed us to measure the amount of free phosphate produced with ATP as a substrate. When ecto-nucleotidase activity was inhibited by ARL67156, pyridoxal phosphate-6-azophenly-2', 4'-disulfonic acid (PPADS), or suramin prior to adding ATP, we found that the rate of phosphate production was significantly reduced by 27%, 21%, and 22%, respectively (P < 0.05). Our second method of measuring ATP metabolism allowed us to measure the amount of adenosine produced following electric field stimulation of the arteriole with and without nucleotidase inhibitors. Surprisingly, we found that adenosine overflow was not attenuated by nucleotidase inhibitors. We concluded that ecto-phosphodieterase/phyrophophatase (E-NPP), ecto-diadenosine polyphosphatase (ApnA), NTPDase1 and 2, and E5NT may be present on the gastrocnemius 1A arteriole and do play a role in ATP metabolism. Between the ages of 8 weeks and 12 weeks, however, overall ATP metabolism may not change.
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PMID:ATP metabolism in skeletal muscle arterioles. 2474 86