Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UNIPROT:P01185 (vasopressin)
 23,126 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

To evaluate the pathomechanism of hyponatremia occasionally seen in Duchenne muscular dystrophy (DMD) on intermittent positive pressure ventilation (IPPV), we performed a comparative study on 26 DMD patients, 7 IPPV in trachetomized intermittent positive pressure ventilation (TIPPV), 6 nasal intermittent positive pressure ventilation (NIPPV), 6 cuirass respirator (CR), and 7 spontaneous breathings (SB). We followed fluctuation of serum sodium levels for several years. Since the serum sodium level was gradually reduced with years on mechanical ventilation, we speculated positive relationship between hyponatremia and antidiuretic hormone (ADH) secretion. Serum sodium levels of 135.6 +/- 2.8 mEq/L in average on IPPV (TIPPV + NIPPV) patients was significantly lower than the levels of 137.8 mEq/L +/- 0.98 on CR patients and 138.8 +/- 0.69 mEq/L on SB patients. Plasma ADH levels in IPPV patients were not reduced when plasma osmolarity was less than 280 mOSm/L. Previous reports showed that ADH was inappropriately secreted only during IPPV with positive end expiratory pressure (PEEP). Ours is the first report of inappropriate secretion of ADH during IPPV without PEEP. It becomes clear that inappropriate ADH secretion is one of the causes of hyponatremia in DMD, though the exact mechanism is not clear. We should bear in mind the change of serum electrolyte level in ventilated DMD patients, especially patients on antidepressant known to release of ADH.
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PMID:[Hyponatremia in Duchenne muscular dystrophy on mechanical ventilation]. 961 71

Dystrophin, utrophin and dystroglycan are present not only in muscle but also in brain. In muscle, they link the extracellular matrix to the cytoskeleton. Their function in brain is not understood. Here we show their presence in the hypothalamo-neurohypophysial system which secretes the neurohormones oxytocin and vasopressin. Using immunocytochemistry, we showed that dystrophins are present in the neurohypophysis of control rats. After water deprivation, immunoreactivity dramatically decreased and appeared in axonal swellings in the hypothalamic tract. Dystrophin immunostaining can be ascribed to dystrophin and/or utrophin as well as the DMD (Duchenne Muscular Dystrophy) gene short products Dp140 and Dp71 as revealed by Western immunoblots of synaptosomes isolated from neurohypophyses of control rats. In synaptosomes isolated from rats under water deprivation, the immunoreactivity entirely disappeared. Further biochemichal characterization of isolated neurosecretory granules (NSG) showed that Dp140 and Dp71 are enriched in the NSG stored in the swellings of the neurohypophysis whereas the NSG of the nerve endings are devoid of these proteins. In addition we observed that the presence of beta-dystroglycan and actin correlates with the presence of dystrophins. Our data favor a direct implication of the dystrophins and/or utrophin, dystroglycan and actin in the neurosecretory processes of the hypothalamo-neurohypophysial system.
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PMID:Dystrophins in neurohypophysial lobe of normal and dehydrated rats: immunolocalization and biochemical characterization. 985 64

Patients with Duchenne muscular dystrophy (DMD) and mdx mice, devoid of dystrophin proteins, show altered ionic homeostasis. To clarify dystrophin's involvement in the central control of osmotic stimuli, we investigated the effect of the disruption of Dp71, the major form of dystrophin in the brain, on the hypothalamoneurohypophysis system (HNHS) osmoregulatory response. Dp71 and Dp140 are the principal DMD gene products in the supraoptic nucleus (SON) and neurohypophysis (NH). They are present in astrocyte and pituicyte end-feet, suggesting involvement in both intrinsic osmosensitivity of the SON and vasopressin (AVP) release from the NH. In Dp71-null mice, the cellular distribution of Dp140 was modified, this protein being detected on the membrane of magnocellular soma. The plasma osmolality of Dp71-null mice was lower than that of wild-type mice under normal conditions, and this difference was maintained after salt loading, indicating a change in the set point for osmoregulation in the absence of Dp71. The increase in AVP levels detected in the SON and NH of the wild-type was not observed in Dp71-null mice following salt loading, and the increase in AVP mRNA levels in the SON was smaller in Dp71-null than in wild-type mice. This suggests that Dp71 may be involved in the functional activity of the HNHS. Its astrocyte end-feet localization emphasizes the importance of neuronal-vascular-glial interactions for the central detection of osmolality. In the SON, Dp71 may be involved in osmosensitivity and definition of the "osmostat," whereas, in the neurohypophysis, it may be involved in fine-tuning AVP release.
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PMID:A deficit of brain dystrophin 71 impairs hypothalamic osmostat. 1965 96

Successful management of hyponatremia in heart failure patients requires a multifaceted approach in order to preserve end-organ function. We describe the novel use of a selective vasopressin receptor antagonist, tolvaptan, for management of hyponatremia in a 17-year-old Caucasian male with severe Duchenne muscular dystrophy, congestive heart failure (CHF), and congenital adrenal hyperplasia. The medical history was significant for recurrent admissions for hyponatremia secondary to adrenal crises, which was also exacerbated by his CHF. After initiation of tolvaptan and its extended administration, he had no further hyponatremia-related admissions and no adverse reactions. The complexity of this combination of conditions is presented, and the efficacy of the drug and the rationale behind the treatment approach is discussed.
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PMID:Novel Use of Tolvaptan in a Pediatric Patient With Congestive Heart Failure Due to Duchenne Muscular Dystrophy and Congenital Adrenal Hyperplasia. 2647 54