نتایج جستجو برای: lipogranulomatosis
تعداد نتایج: 79 فیلتر نتایج به سال:
This paper describes an approach to the analysis of time-dependent systems which combines discrete-event simulation and model-checking techniques. The approach rests on Merlin and Farber’s Time Petri Nets (TimePNs) and is supported by a Java toolbox TPN Designer which enables graphical modelling, simulation and translation into UPPAAL/Timed Automata, for exhaustive state space verification, of ...
1. Barberá JA, Escribano P, Morales P, Gómez MA, Oribe M, Martínez A, et al. Estándares asistenciales en hipertensión pulmonar. Rev Esp Cardiol. 2008;61:170-84. 2. Angell M. Is academic medicine for sale? N Engl J Med. 2000;342:1516-8. 3. Bodenheimer T. Uneasy alliance —clinical investigators and the pharmaceutical industry. N Engl J Med. 2000;342:1539-44. 4. Campbell EG, Weissman JS, Vogeli C,...
The problem of classification in terms of a finite list of invariants is a fundamental question of knot theory. In 1970, Levine I-L] gave an algebraic classification of n-dimensional knots in S n+2 bounding r-connected Seifert surfaces, where 2 r+ 1 = n, n> 3. Levine showed that the only invariant which determines the isotopy type of such knots is the homology Seifert pairing considered up to S...
Farber disease (FD) is a severe inherited disorder of lipid metabolism characterized by deficient lysosomal acid ceramidase (ACDase) activity, resulting in ceramide accumulation. Ceramide and metabolites have roles in cell apoptosis and proliferation. We introduced a single-nucleotide mutation identified in human FD patients into the murine Asah1 gene to generate the first model of systemic ACD...
This paper describes an approach to the analysis of time-critical systems which combines discreteevent simulation and model-checking techniques. The approach rests on Merlin and Farber’s Time Petri Nets (TimePNs) and is supported by a Java toolbox −TPN/DESIGNER− which enables graphical modelling and simulation of modular TimePN models. The toolbox also allows translation of a TimePN model into ...
The crystal structure of APE2540, the putative trans-editing enzyme ProX from Aeropyrum pernix K1, was determined in a high-throughput manner. The crystal belongs to the monoclinic space group P2(1), with unit-cell parameters a = 47.4, b = 58.9, c = 53.6 A, beta = 106.8 degrees. The structure was solved by the multiwavelength anomalous dispersion method at 1.7 A and refined to an R factor of 16...
We study cobordisms and cobordisms rel boundary of PL locally-flat disk knots Dn−2 ↪→ Dn. Any two disk knots are cobordant if the cobordisms are not required to fix the boundary sphere knots , and any two even-dimensional disk knots with isotopic boundary knots are cobordant rel boundary. However, the cobordism rel boundary theory of odd-dimensional disk knots is more subtle. Generalizing resul...
We have reported previously that the ceramidase from Pseudomonas aeruginosa AN17 isolated from a patient with atopic dermatitis requires detergents for hydrolysis of ceramide (Cer) [Okino, Tani, Imayama and Ito (1998) J. Biol. Chem. 273, 14368--14373]. In the present study, we report that some glycerophospholipids strongly activated the hydrolysis of Cer by Pseudomonas ceramidase in the absence...
Introduction The problem of disparities in medical care and clinical outcomes affecting people of color and those living in poverty is convincingly documented by a growing body of evidence from many fields of medicine (1). Cancer care is no exception (2, 3). An often neglected question is what academic health centers can and should do to correct this problem. Clearly, the academic centers are n...
Farber disease caused by acid ceramidase deficiency is characterised by a triad of painful and swollen joints, subcutaneous nodules, and laryngeal involvement. A one year old female with overlapping features of the classical and type 5 variants is reported. Sialuria and elevated plasma chitotriosidase were unusual findings. A novel mutation of the ASAH 1 gene was detected from DNA extracted fro...
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