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21.
Mutational analysis of the SOX9 gene in campomelic dysplasia and autosomal sex reversal: lack of genotype/phenotype correlations 总被引:9,自引:1,他引:9
Meyer J; Sudbeck P; Held M; Wagner T; Schmitz ML; Bricarelli FD; Eggermont E; Friedrich U; Haas OA; Kobelt A; Leroy JG; Van Maldergem L; Michel E; Mitulla B; Pfeiffer RA; Schinzel A; Schmidt H; Scherer G 《Human molecular genetics》1997,6(1):91-98
It has previously been shown that, in the heterozygous state, mutations in
the SOX9 gene cause campomelic dysplasia (CD) and the often associated
autosomal XY sex reversal. In 12 CD patients, 10 novel mutations and one
recurrent mutation were characterized in one SOX9 allele each, and in one
case, no mutation was found. Four missense mutations are all located within
the high mobility group (HMG) domain. They either reduce or abolish the
DNA-binding ability of the mutant SOX9 proteins. Among the five nonsense
and three frameshift mutations identified, two leave the C-terminal
transactivation (TA) domain encompassing residues 402-509 of SOX9 partly or
almost completely intact. When tested in cell transfection experiments, the
recurrent nonsense mutation Y440X, found in two patients who survived for
four and more than 9 years, respectively, exhibits some residual
transactivation ability. In contrast, a frameshift mutation extending the
protein by 70 residues at codon 507, found in a patient who died shortly
after birth, showed no transactivation. This is apparently due to
instability of the mutant SOX9 protein as demonstrated by Western blotting.
Amino acid substitutions and nonsense mutations are found in patients with
and without XY sex reversal, indicating that sex reversal in CD is subject
to variable penetrance. Finally, none of 18 female patients with XY gonadal
dysgenesis (Swyer syndrome) showed an altered SOX9 banding pattern in SSCP
assays, providing evidence that SOX9 mutations do not usually result in XY
sex reversal without skeletal malformations.
相似文献
22.
Santoro Giorgio Piccirilli Manolo Chiarella Vito Greco Nicoletta Berra Luigi Valentino Santoro Antonio 《Neurosurgical review》2021,44(4):1977-1985
Neurosurgical Review - Capillary hemangiomas (CHs) of the central nervous system represent a rare diagnosed pathology. CHs are benign vascular tumors whose most common manifestations are dermal and... 相似文献
23.
Ricciardi Luca Stifano Vito Pucci Resi Stumpo Vittorio Montano Nicola Della Monaca Marco Lauretti Liverana Olivi Alessandro Valentini Valentino Sturiale Carmelo Lucio 《Neurosurgical review》2021,44(1):153-161
Neurosurgical Review - The surgical injury of the intracranial portion of the facial nerve (FN) is a severe complication of many skull base procedures, and it represents a relevant issue in terms... 相似文献
24.
The characteristics of facial nerve electromyography at various levels of neuromuscular blockade are unclear. Partial blockade is well known to facilitate anesthetic safety and management. However, the use of neuromuscular blockage in many skull base procedures is avoided to allow intraoperative facial nerve monitoring.We studied the influence of various levels of neuromuscular blockade on facial nerve stimulation in the New Zealand white rabbit. The facial nerve was exposed in the middle ear of six rabbits. Using electromyographic-type facial nerve monitor, we recorded the facial electromyography signals in these rabbits at increasing levels of vecuronium-induced neuromuscular blockade. All animals demonstrated reliable facial electromyography response at all levels of partial neuromuscular blockade (P < .02). Five of the six animals could be monitored throughout complete blockade. These results clearly demonstrate that rabbit facial electromyography monitoring is possible under neuromuscular blockade. The effect of neuromuscular blockers on facial electromyography monitoring deserves further study, as partial blockade would greatly facilitate the management of anesthesia in otologic, neurotologic, and skull base surgery. 相似文献
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G. Germano' MD A. Caparra MD S. Valentino MD U. Germano' MD F. Coia MD M. Cassone-Faldetta MD G. De Mattia MD A. Santucci MD F. Balsano MD 《Clinical autonomic research》1993,3(4):249-254
The 24 h periodic pattern of blood pressure was studied in 44 patients with diabetes mellitus (14 type 1, 30 type 2; mean duration of disease 6.5 ± 1.8 years) in good metabolic control but with abnormal cardiovascular reflex responses; of these 21 were normotensive and 23 hypertensive. All had abnormal responses to at least two out of four tests: deep breathing, lying to standing, Valsalva manoeuvre and postural hypotension. Two sex- and agematched groups, consisting of 20 normotensive and 20 hypertensive diabetic patients without dysautonomia, were studied as controls. Each patient underwent ambulatory blood pressure monitoring for at least 24 h, using an auscultatory automatic device. Data were analysed using the sum of three periodic functions (Fourier partial sum). In the diabetic normotensive groups, the absolute blood pressure fell to its night-time minimum more rapidly, and increased to its morning maximum more slowly, in those with abnormal cardiovascular reflexes than in the controls (nightly blood pressure decrease –5.8/–4.7 vs. –3.8/–4.0 mmHg/h; increase 4.7/3.6 vs. 5.9/6.1 mmHg/h). The same behaviour was found in both hypertensive groups but the amplitude of the differences was more marked (blood pressure nocturnal decrease –7.7/–7.1 vs. –4.3/–3.9 mmHg/h; increase 3.2/2.1 vs. 5.8/4.3 mmHg/h). This analysis of 24 h ambulatory blood pressure data may be of value in diagnosis and evaluation of autonomic deficits. 相似文献
28.
As Stock and LeFroy conclude, a RM program lends structure and direction to management. Consistent delivery of high-quality service requires that hundreds of individuals perform numerous roles with a value-added service orientation. Jan Carlzon, the Director of Scandinavian Airlines and the person responsible for its remarkable turnaround, has the proper perspective: "We don't seek to be one thousand percent better at any one thing. We seek to be one percent better at one thousand things." Risk management is one factor in the development of a service-oriented health care culture that leads to sustained profitability through a stable patient base. The development of a RM program for a private dental office will lay the groundwork for a higher goal; a demonstrable, verifiable quality of care throughout the practice. 相似文献
29.
Balloon angioplasty is a safe and effective alternative to surgical reconstruction or bypass in selected patients with symptomatic iliofemoral and popliteal atherosclerosis. The current literature suggests an expanding role for balloon angioplasty in patients with symptomatic tibioperoneal disease as well as upper extremity claudication and subclavian steal syndrome. Newer percutaneous devices such as atherectomy, intravascular stents, and laser angioplasty may further broaden the indications for percutaneous intervention by improving their safety and long-term benefit. The latest advances in percutaneous intervention for peripheral vascular disease are reviewed. 相似文献
30.
Aldo Becciolini Manuela Balzi Paola Faraoni Elena Tisti Giorgia Zappoli Thyrion Valentino Giach Luca Bandettini Christopher S. Potten 《Acta oncologica (Stockholm, Sweden)》1998,37(1):65-71
Cell kinetics parameters have been analysed in colonic mucosa at different distances from a tumour in patients with colon carcinoma. Total cell number (TCN), H thymidine labelling index (TLI), mitotic index (MI), Goblet cell index (GCI) and the distribution of labelled cells along the crypt column (cell position frequency plot) were determined in well-aligned crypts. Total cell number, GCI and the labelled cell position frequency plots were similar in different samples from the same individual. A negative linear correlation between TCN and TLI was observed. The analysis of the cell position plots showed two patterns 1) with a high concentration in the bottom fifth of the crypt and 2) with frequent labelled cells at high positions. Whereas a negative correlation between overall TLI and the percent contribution to the TLI of the lowermost fifth was seen, the correlation was positive for the next 3 fifths and labelling was absent in the last part of the crypt. 相似文献