نتایج جستجو برای: zellweger syndrome
تعداد نتایج: 622015 فیلتر نتایج به سال:
A method is proposed for arbitrarily engineering the high-order harmonic generation phase achieved by shaping a laser pulse and employing xuv light or x rays for ionization. This renders the production of bandwidth-limited attosecond pulses possible while avoiding the use of filters for chirp compensation. By adding the first 8 Fourier components to a sinusoidal field of 1016 W/cm2, the bandwid...
J. Taprogge, A. Jungclaus, H. Grawe, S. Nishimura, P. Doornenbal, G. Lorusso, G. S. Simpson, P.-A. Söderström, T. Sumikama, Z. Y. Xu, H. Baba, F. Browne, N. Fukuda, R. Gernhäuser, G. Gey, N. Inabe, T. Isobe, H. S. Jung, D. Kameda, G. D. Kim, Y.-K. Kim, I. Kojouharov, T. Kubo, N. Kurz, Y. K. Kwon, Z. Li, H. Sakurai, H. Schaffner, K. Steiger, H. Suzuki, H. Takeda, Zs. Vajta, H. Watanabe, J. Wu, A...
Peroxisomes are subcellular organelles catalyzing a number of indispensable functions in cellular metabolism. The importance of peroxisomes is stressed by the existence of an expanding number of genetic diseases in which there is an impairment of one or more peroxisomal functions. The prototype of this group of diseases is the cerebro-hepato-renal syndrome of Zellweger (ZS), first described as ...
Zellweger syndrome, neonatal adrenoleukodystrophy, infantile Refsum's disease, and classical rhizomelic chondrodysplasia punctata are lethal genetic disorders caused by defects in peroxisome biogenesis. We report here a characterization of the peroxisomal matrix protein import capabilities of fibroblasts from 62 of these peroxisome biogenesis disorder patients representing all ten known complem...
Zellweger syndrome (ZS) is a peroxisomal disorder with a multiple congenital anomalies, characterized by stereotypical facies, profound hypotonia, organ involvement including cerebral, retinal, hepatic, and renal. Herein, a 3-month-old female with ZS is presented who was referred because of increased liver enzymes (subclinical hepatitis), which was detected in work-up of her neck cyst, severe h...
MEGA-3 FATTY ACIDS, found mainly in fish oils, are said to be beneficial in conditions from Alzheimer disease to Zellweger syndrome. Much of the evidence is circumstantial and indirect, so if and how to use them is still open to question. We do however have solid and compelling evidence that two longchain omega-3 fatty acids—eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA)—help preven...
The intracellular localization of Cu,Zn superoxide dismutase (superoxide:superoxide oxidoreductase, EC 1.15.1.1) has been examined by immunofluorescence using four monoclonal anti-Cu,Zn superoxide dismutase antibodies raised against a recombinant human Cu,Zn superoxide dismutase derivative produced and purified from Escherichia coli. Colocalization with catalase, a peroxisomal matrix enzyme, wa...
Since the first report by Lejeune, Gautier, and Turpin in 1959 of the findings of trisomy 2I and a chromosome number of 47 in Down's syndrome (mongolism), several different chromosomal patterns have been described in patients with this disorder. One of these is chromosome mosaicism. In such cases, the patient may lack some of the features characteristic of the syndrome-and indeed some subjects ...
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