Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P50583 (asymmetrical)
 12,197 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

ACTH increases the basal steroidogenic activity of cultured adrenocortical tumor cells, whereas moderate-high doses of cytochalasin B (CB) inhibit both basal and ACTH-induced steroidogenesis. Previous ultrastructural studies have revealed that ACTH rearranges microfilaments in these adrenal cells, whereas CB causes microfilaments to aggregate into felt-like masses. It has been postulated that the ACTH effects may facilitate organelle motility and increase organelle interactions that are required for steroid biosynthesis, and that the CB-created "foci" may impede or prevent the organelle meetings. To shed light on these possibilities, we have employed 16 mm cinemicrography of unstimulated adrenal tumor cells and cells incubated for 1-2 h with ACTH (10 mU/ml), or low (10 micrograms/ml), or high (50 micrograms/ml) doses of CB. ACTH caused initial increases in membrane ruffling and a "flurry" of particle (organelle) activity above that seen in unstimulated cells. The stimulated cells then retracted from each other and began their characteristic "rounding up" in response to the hormone. Particles appeared to move towards the nucleus, and in fully-rounded cells were extremely congested. Steroid production rose several fold above basal levels. CB10 produced slight-marked cell convexities, nearly stopped particle motility and inhibited steroid production moderately. CB50 produced an asymmetrical, spidery cell form, stopped membrane ruffling and particle motility and abolished steroidogenesis. After a washout of CB50, particle motility resumed nearly immediately. Our CB data indicate that associations between particles, presumably between mitochondria and various sources of cholesterol, are prerequisite for basal steroidogenesis in the adrenal tumor cells. In ACTH-stimulated cells, increases in steroid output correspond with increased opportunities for particle associations. These opportunities appear to arise directly or indirectly from ACTH effects on microfilaments. The responses of microfilaments to the hormone may be particularly intense in tumorous forms. By these means, the cells may express their differentiated function, although their cytoplasm has a distinctly unspecialized appearance.
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PMID:Cinemicrographic observations of cultured adrenocortical tumor cells. Dynamic responses to ACTH and cytochalasin B. 287 79

A multimodality radioimmunological and clinicobiochemical study showed an increase in the hydrocortisone level and a decrease in the ACTH level in breast cancer patients. Stage I, following radical mastectomy (RM). A simultaneous increase in the hydrocortisone, ACTH and aldosterone level after RM, chemotherapy, hormone therapy was noted in all patients with breast cancer, Stages II and III, with initially lowered hydrocortisone and aldosterone levels and an elevated ACTH level. After RM and radiotherapy the level of hydrocortisone decreased, that of ACTH and aldosterone and renin activity increased. Scintigraphically in Stage I breast cancer the adrenals were enlarged, after RM in some cases they remained enlarged, in other cases they returned to normal. Enlarged adrenals with a lowered contrast and enhanced obscurity of the contours were observed in Stage II and III breast cancer. After therapy the adrenals remained asymmetrical with a low contrast.
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PMID:[Combined examination of the hypophysis-adrenal cortex system in breast cancer]. 402 50

A 45-yr-old woman presented with a 2-hr history of Cushing's syndrome. Baseline cortisol and ACTH levels were elevated and lacked a normal circadian rhythm. Dynamic testing of her pituitary-adrenal function revealed positive responses to metyrapone and synthetic ACTH and suppression with high doses of dexamethasone. The finding of asymmetrical adrenal uptake of 6-[131I]methyl-19-nor-cholesterol and a tumor image in the right adrenal on venography led to the preoperative diagnosis of pituitary ACTH-dependent adrenal hyperfunction coexisting with a right adrenal adenoma. Surgical resection of the right adrenal demonstrated a solitary 39-g nodule, 4 cm in diameter. There were no additional nodules present in the surrounding cortex. When incubated in vitro, both the adenoma and the surrounding tissue were functional and responsive to ACTH. This was in contrast to the lack of response to ACTH in vitro of similar tissues removed from two other patients with ACTH-independent adrenal adenomas. We suggest that this case represents a transition between pituitary ACTH-dependent bilateral adrenocortical hyperfunction and solitary adrenal adenoma.
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PMID:Coexistence of pituitary adrenocorticotropin-dependent Cushing's syndrome with a solitary adrenal adenoma. 624 39

Scintigraphy was used in 66 patients with biochemically demonstrated hyperactivity of the adrenal cortex in order to determine the nature and site of the lesions. In cases of hypercortisolism, uptake was bilateral in 12 patients with Cushing's disease, unilateral in 7 patients with malignant or non-malignant tumours, and absent in 3 cases of large malignant tumours. In cases of hyperaldosteronism, scintigraphy performed during dexamethasone-induced ACTH suppression showed distinctly asymmetrical uptake in 13 patients with Conn's adenoma (confirmed by surgery as being on the good uptake side in 10 patients), symmetrical in 20 patients with biochemical findings indicating bilaterality, and intermediate in 9 patients. There was no false positive diagnosis of tumour. Scintigraphy appears to be of considerable value for locating adrenocortical lesions, especially small tumours.
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PMID:[Adrenocortical scintigraphy with 131I-19-iodocholesterol. Indications and results in 66 cases of hypercortisolism and hyperaldosteronism (author's transl)]. 722 Mar 32

To describe the poorly known characteristics of epilepsy during infancy in focal cortical dysplasia (FCD), one of the most frequent cause of infantile epilepsy. All 28 patients with FCD referred to two specialized centres were retrospectively studied regarding seizure characteristics, psychomotor evaluation, and response to medical and surgical treatment. All patients presented with early partial seizures. Semiology, but not the age of onset, depended on the topography of the dysplasia, with abnormal eye movements in all cases of posterior FCD. Eleven patients also developed infantile spasms (IS), mainly asymmetrical. IS were easily controlled with Vigabatrin or ACTH, but no partial seizures could be medically controlled except in one patient. All patients except one had abnormal neuropsychological findings. Fifteen patients had surgery, eight became seizure free, and seven were significantly improved regarding psychomotor development. Very early and refractory partial seizures, but easily controlled IS are the main characteristics of FCD in infancy. Only the focal ictal semiology may help differentiate the localization of FCD. Its intrinsic epileptogenicity could sustain this clinical pattern. Since the chances for medical control and normal neurodevelopment are poor, surgical treatment should be considered early in infants with FCD.
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PMID:Characteristics of epilepsy in focal cortical dysplasia in infancy. 1235 Mar 89

The following aspects are reviewed: Does the aetiology influence the outcome of infantile spasms? Does the treatment influence the outcome? Can the outcome be predicted? Can we improve the prognosis? Favourable factors are the following: cryptogenic aetiology, age at onset > or =4 months, absence of atypical spasms and partial seizures, and absence of asymmetrical EEG abnormalities, short treatment lag, and an early and sustained response to treatment. Not only patients with a cryptogenic aetiology have a favourable outcome. We can already at the first clinical evaluation tell the parents if the prognosis looks favourable. The final goal of the treatment is improved mental outcome. Steroids and vigabatrin are the first-line drugs for infantile spasms in Europe. In a prospective study from the United Kingdom short-term outcome was better with hormonal than with vigabatrin therapy (tuberous sclerosis excluded). However, the numbers of patients who were seizure-free at 3-4 months in different studies have been very similar. Moreover, an early response to treatment seems to be of predictive value for the cognitive outcome in children with cryptogenic spasms. The long-term outcome is known only after hormonal therapy. The side effects of steroids are usually treatable and reversible. In Finland ACTH therapy is given at the minimum effective dose and for the minimum effective time with minimal side effects. The risks of VGB are irreversible visual field defects. As of yet there is no method to examine the visual fields in patients with infantile spasms. Early treatment of infantile spasms seems to be important. Prevention of infantile spasms with some aetiological groups might be possible.
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PMID:Favourable prognostic factors with infantile spasms. 1936 67

We describe the EEG findings of an infant with early-onset epileptic encephalopathy with mutation of the KCNQ2 gene and a family history of neonatal seizures. The infant presented with multifocal drug-resistant seizures with onset during the third day of life. Family history was positive for early-onset neonatal seizures. Metabolic screening and neuroimaging were negative. Direct sequencing of KCQN2 from both the mother and child revealed a heterozygous cytosine-to-guanine mutation (Dedek et al., 2003). Interictal EEG showed a very discontinuous pattern which evolved towards a defined burst-suppression pattern during sleep and a multifocal, random, attenuation pattern during wakefulness. Focal, tonic seizures with head deviation, sometimes followed by asynchronous and asymmetrical clonic jerks, eyelid myoclonias, and polypnoea, were recorded. Ictal EEG was characterised by focal, low-voltage, fast activity, followed by recruiting theta rhythms and bilateral, focal, spike-wave complexes, alternatively localised to one hemisphere and subsequently diffusing to the other. ACTH therapy was introduced, resulting in a significant improvement in EEG activity and gradual reduction in seizure frequency, with cessation at age 13 weeks. [Published with video sequences].
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PMID:Video/EEG findings in a KCNQ2 epileptic encephalopathy: a case report and revision of literature data. 2377 9