نتایج جستجو برای: zellweger syndrome
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the set T1 + T2 + T3 + . . . + Tn. Let K denote the set of all points X of M such that X can be joined to A by a connected subset of M which contains no point outside of C and let E denote the sum of the sets H and K. Let D denote the complementary domain of E which contains B and let L denote the boundary of D. Since K is2 a continuous curve and each Ti is3 a continuous curve, therefore, E is ...
College of Agriculture, University of Illinois. The senior author is alone responsible for the manuscript and any views or shortcomings it may contain. 2 Detlefsen, A., These PROCUSDINGS, 6, 1920 (663-670). 8Weinstein, A., Genetics, 3, 1918 (135-174). 4Sturtevant, A. H., These PROCERDINGS, 7, 1921 (181-183). 6 Sturtevant, A. H., Carnegie Institution, Washington, Publ., No. 278, 1919 (305-341). ...
has a nonzero integer solution for every > 0. If some Qi is rational and is small enough then for x ∈ Zs the inequality |Qi(x)| < is equivalent to the equation Qi(x) = 0. Hence if all forms are rational then for sufficiently small the system (1.1) reduces to a system of equations. In this case W. Schmidt [10] proved the following result. Recall that the real pencil generated by the forms Q1, . ...
2 For a detailed account of the methods reference may be made to C. Ramsauer, Ann. Physik., 72, p. 345, 1923, and Jahrb. Radioaktivitdt, 19, p. 345, 1923; R. B. Brode, Physic. Rev., 25, p. 636, 1925, and Proc. of the Royal Soc., 109, p. 397, 1925. 3 F. Zwicky, Physik. Zeitschr., 24, p. 171, 1923. 4E. C. Stoner, Phil. Mag., 48, p. 719, 1924. 5 F. Hund, Zs. Physik, 13, p. 241, 1923; G. Wentzel, I...
Here, we present the whole-genome sequence of an environmental Gram-negative Alishewanella aestuarii strain (HH-ZS), isolated from the hyperalkaline contaminated soil of a historical lime kiln in Buxton, United Kingdom.
The presence of peroxisomal membrane ghosts was examined in liver biopsies from eleven patients presenting the clinical and biochemical picture of a generalized peroxisomal disorder (Zellweger syndrome, neonatal adrenoleukodystrophy, infantile Refsum disease and variants of these syndromes). A polyclonal antibody raised against the membrane of human liver peroxisomes and recognizing a 43 kDa pe...
Human peroxisome biogenesis disorders are lethal genetic diseases in which abnormal peroxisome assembly compromises overall peroxisome and cellular function. Peroxisomes are ubiquitous membrane-bound organelles involved in several important biochemical processes, notably lipid metabolism and the use of reactive oxygen species for detoxification. Using cultured cells, we systematically character...
Peroxisomal matrix protein import requires the action of two AAA ATPases, PEX1 and PEX6. Mutations in either the PEX1 or PEX6 gene are the most common cause of the lethal neurologic disorders Zellweger syndrome, neonatal adrenoleukodystrophy, and infantile Refsum disease and account for disease in 80% of all such patients. We report here that overexpression of PEX6 can suppress the phenotypes o...
Many cell surface proteins in mammalian cells are anchored to the plasma membrane via glycosylphosphatidylinositol (GPI). The predominant form of mammalian GPI contains 1-alkyl-2-acyl phosphatidylinositol (PI), which is generated by lipid remodeling from diacyl PI. The conversion of diacyl PI to 1-alkyl-2-acyl PI occurs in the ER at the third intermediate in the GPI biosynthetic pathway. This l...
Functional peroxisome deficiency, as encountered in Zellweger syndrome, causes a specific impairment of neuronal migration. Although the molecular mechanisms underlying the neuronal migration defect are at present unknown, the excess of very long chain fatty acids in brain, a consequence of peroxisomalbeta-oxidation deficiency, has often been hypothesized to play a major role. The purpose of th...
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