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排序方式: 共有3788条查询结果,搜索用时 31 毫秒
1.
Bela Anand-Apte Jennifer R. Chao Ruchira Singh Heidi Stöhr 《Journal of neuroscience research》2019,97(1):88-97
Sorsby fundus dystrophy (SFD), an autosomal dominant, fully penetrant, degenerative disease of the macula, is manifested by symptoms of night blindness or sudden loss of visual acuity, usually in the third to fourth decades of life due to choroidal neovascularization (CNV). SFD is caused by specific mutations in the Tissue Inhibitor of Metalloproteinase-3, (TIMP3) gene. The predominant histo-pathological feature in the eyes of patients with SFD are confluent 20–30 m thick, amorphous deposits found between the basement membrane of the retinal pigment epithelium (RPE) and the inner collagenous layer of Bruch's membrane. SFD is a rare disease but it has generated significant interest because it closely resembles the exudative or “wet” form of the more common age-related macular degeneration (AMD). In addition, in both SFD and AMD donor eyes, sub-retinal deposits have been shown to accumulate TIMP3 protein. Understanding the molecular functions of wild-type and mutant TIMP3 will provide significant insights into the patho-physiology of SFD and perhaps AMD. This review summarizes the current knowledge on TIMP3 and how mutations in TIMP3 cause SFD to provide insights into how we can study this disease going forward. Findings from these studies could have potential therapeutic implications for both SFD and AMD. 相似文献
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W. Nörenberg Ernst Schöffel Bela Szabo Klaus Starke 《Naunyn-Schmiedeberg's archives of pharmacology》1997,356(2):159-165
The aim of the study was to subclassify the soma-dendritic α2-autoreceptors in the locus coeruleus (LC) of the rat by means of antagonists. To this end, the frequency of spontaneous action
potentials was recorded extracellularly from single LC neurones in brain slices. The neurones fired spontaneously at an average
rate of 1 Hz. The selective α2-adrenoceptor agonist 5-bromo-6-(2-imidazolin-2-ylamino)-quinoxaline (UK 14,304) and noradrenaline decreased the action potential
discharge with IC50 values of 5 and 510 nM, respectively. The concentration-inhibition curves of UK 14,304 and noradrenaline were shifted to the
right by phentolamine (0.15 μM) and rauwolscine (0.15 μM) but not by prazosin (1 μM). Apparent K
d values of phentolamine were 17 nM (against UK 14,304) and 20 nM (against noradrenaline). Apparent K
d values of rauwolscine were 47 nM (against UK 14,304) and 70 nM (against noradrenaline). (+)-Oxaprotiline (1 μM) suppressed
the firing of the neurones within 10 to 33 min. In the continued presence of oxaprotiline, phentolamine and rauwolscine restored
firing with EC50 values of 120 and 250 nM, respectively. Prazosin (1 μM) again was ineffective. All three antagonist affinity estimates –
against UK 14,304, exogenous noradrenaline and endogenous noradrenaline (that accumulates in the extracellular space in the
presence of oxaprotiline) – yield an affinity order phentolamine > rauwolscine >> prazosin, prazosin being ineffective even
at a concentration of 1 μM. These findings identify the soma-dendritic α2-autoreceptors of the LC as the rat variant of the α2A/D-adrenoceptor, i.e. α2D. Not only presynaptic but also soma-dendritic α2-autoreceptors may at least predominantly be α2A/D throughout the nervous system.
Received: 3 March 1997 / Accepted: 21 April 1997 相似文献
4.
Samir Kumar Praharaj mbbs md dpm Nishant Goyal mbbs md dpm Sukanto Sarkar mbbs md dpm Dhruv Bagati mbbs Prakriti Sinha mp hil Vinod Kumar Sinha mbbs md dpm 《Psychiatry and clinical neurosciences》2009,63(2):230-234
Reported herein is a case of obsessive–compulsive disorder with persistent and distressing musical obsessions along with other symptoms. Advanced source analysis of electroencephalographic data indicated high spectral power over the bifrontal region. The musical symptoms were resistant to pharmacotherapy but there was some reduction in frequency and duration of musical obsessions with thought-stopping technique. 相似文献
5.
Fibrillary glomerulonephritis and immunotactoid glomerulopathy. 总被引:6,自引:0,他引:6
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Bela Prajapaty Bharat Shah 《Indian journal of otolaryngology and head and neck surgery》1997,49(4):391-393
The pyriform sinus fistula is rare congenital anomaly of either third or fourth branchial pouch, which most frequently presented itself by recurrent episodes of neck abscess for long time. Majority of patients with this anomaly presented with symptoms before 10 years and it is more common than has previously been commonly found on leftside (93%). Clinically high index of suspicion, cannulation of the tract under general anaesthesia, Direct laryngoscopy and fistulogram have freequently been successful in identifying the presence of these embryological remnants. Surgical exploration of sinus tract with its total excision, guided with cannula inside its lumen is the definitive treatment. 相似文献
8.
Bela Blasszauer 《The Hastings Center report》1986,16(3):25-27
Medical ethics in Hungary has finally moved beyond arguments over tipping and bribes to discussions of euthanasia, confidentiality, truth-telling and informed consent. Ethics committees have been formed at the local and national level, and medical schools are beginning to take seriously the systematic teaching of medical ethics. In some quarters, however, old attitudes persist. Among older doctors paternalism reigns supreme, and lawmakers continue to ignore international ethical guidelines. 相似文献
9.
V Bhattacharya G R Reddy Sunish Goyal Umesh Kumar 《Journal of plastic, reconstructive & aesthetic surgery》2007,60(8):892-897
Defects involving the distal leg and foot are frequently encountered following various aetiological factors. Paucity of local tissue causes surgeons to resort to the retrograde peninsular flap, the cross leg flap or the free flap. With specific knowledge of perforators, the fasciocutaneous flap from the calf area can be transferred to the defect in a single stage based on skeletonised distal perforators. The surgical anatomy, flap planning and procedure have been detailed. Nineteen patients were treated during the period 1995 to 2005. The perforators were identified preoperatively by audio Doppler. The flaps were marked and dissected proximal to the defect skeletonising the distal perforators under loupe magnification and transferred to the defect in a single stage. The donor site was skin grafted. Out of 19 cases, 16 flaps healed uneventfully, one flap necrosed completely and in two cases there was marginal necrosis. The cases were followed up for 2-10 years with an average of 6 years. With detailed knowledge of perforators one can safely reconstruct distal moderate-size defects of the lower limb in a single stage, thus having the benefits of free tissue transfer without resorting to microsurgery. This technique has proved to be an advancement in the reconstructive repertoire allowing flaps of non conventional dimensions to be perfused by skeletonised perforators. 相似文献
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