679 research outputs found
Structure and evolution of the magnetochrome domains: no longer alone
Magnetotactic bacteria (MTB) can swim along Earth's magnetic field lines, thanks to the alignment of dedicated cytoplasmic organelles. These organelles, termed magnetosomes, are proteolipidic vesicles filled by a 35–120 nm crystal of either magnetite or greigite. The formation and alignment of magnetosomes are mediated by a group of specific genes, the mam genes, encoding the magnetosome-associated proteins. The whole process of magnetosome biogenesis can be divided into four sequential steps; (i) cytoplasmic membrane invagination, (ii) magnetosomes alignment, (iii) iron crystal nucleation and (iv) species-dependent mineral size and shape control. Since both magnetite and greigite are a mix of iron (III) and iron (II), iron redox state management within the magnetosome vesicle is a key issue. Recently, studies have started pointing out the importance of a MTB-specific c-type cytochrome domain found in several magnetosome-associated proteins (MamE, P, T, and X). This magnetochrome (MCR) domain is almost always found in tandem, and this tandem is either found alone (MamT), in combination with a PDZ domain (MamP), a domain of unknown function (MamX) or with a trypsin combined to one or two PDZ domains (MamE). By taking advantage of new genomic data available on MTB and a recent structural study of MamP, which helped define the MCR domain boundaries, we attempt to retrace the evolutionary history within and between the different MCR-containing proteins. We propose that the observed tandem repeat of MCR is the result of a convergent evolution and attempt to explain why this domain is rarely found alone
Subset currents on free groups
We introduce and study the space of \emph{subset currents} on the free group
. A subset current on is a positive -invariant locally finite
Borel measure on the space of all closed subsets of consisting of at least two points. While ordinary geodesic currents
generalize conjugacy classes of nontrivial group elements, a subset current is
a measure-theoretic generalization of the conjugacy class of a nontrivial
finitely generated subgroup in , and, more generally, in a word-hyperbolic
group. The concept of a subset current is related to the notion of an
"invariant random subgroup" with respect to some conjugacy-invariant
probability measure on the space of closed subgroups of a topological group. If
we fix a free basis of , a subset current may also be viewed as an
-invariant measure on a "branching" analog of the geodesic flow space for
, whose elements are infinite subtrees (rather than just geodesic lines)
of the Cayley graph of with respect to .Comment: updated version; to appear in Geometriae Dedicat
Aromatic L-Amino Acid Decarboxylase Deficiency Is a Cause of Long-Fasting Hypoglycemia
Objective/Context:
Long-fasting hypoglycemia in children may be induced by neurotransmitter disorders.
Case Report:
A 5-year-old girl with a medical history of chronic diarrhea presented three episodes of severe hypoglycemia (20 mg/dL) between ages 3 and 5 years. She became pale and sweaty with hypothermia (33.5°C), bradycardia (45 bpm), and acidosis and presented a generalized seizure. During the 17-hour fast test performed to determine the etiology of her hypoglycemia, insulin and C-peptide were appropriately low, and human GH, IGF-I, cortisol, amino acids, and acylcarnitines were in the usual range for fasting duration. However, the presence of vanillactic and vanilpyruvic acids in urine led us to investigate the metabolism of dopamine and serotonin in the cerebrospinal fluid. Indeed, these results indicated an aromatic L-amino acid decarboxylase deficiency that impairs the synthesis of serotonin, dopamine, and catecholamines. The diagnosis was confirmed by the low aromatic L-amino acid decarboxylase (AADC) enzyme activity in plasma (5 pmol/min/mL; reference value, 20–130) and the presence of two heterozygous mutations, c.97G>C (p.V33L, inherited from her father) and c.1385G>C (p.R462P, inherited from her mother) in the DCC gene. She was supplemented with pyridoxine and raw cornstarch (1 g/kg) at evening dinner to reduce the night fast. The episodes of hypoglycemia and the chronic diarrhea were suppressed.
Conclusion:
Here is the first case report of long-fasting hypoglycemia due to a nontypical AADC deficiency. Hypoglycemia was severe, but the other neurological clinical hallmarks present in AADC-deficient patients were mild to moderate. Thus, neurotransmitter disorders should be considered in any patients presenting hypoglycemia with urine excretion of vanillactic acid
The effect of ECRH on the electron velocity distribution function
Electron cyclotron resonance heating (ECRH) is a mature technology that has progressed constantly over a period of forty years, particularly as a tool in magnetic confinement fusion. As with other heating methods, this technique has seen a steady increase in the sophistication of its applications, from bulk heating through profile tailoring and finally to distribution function engineering. By comparison with other techniques, ECRH presents the significant advantages of good coupling, localized power deposition, easy launching and precise directionality. This paper reviews some recent applications related to third harmonic ECRH and highlights the role of the relaxation dynamics of suprathermal electrons, both in real space and in velocity space, in regulating the overall effect of ECRH on fusion plasmas. A technique for direct visualization of these relaxation phenomena, using modulated ECRH, is described and demonstrated
Triangulations and Severi varieties
We consider the problem of constructing triangulations of projective planes
over Hurwitz algebras with minimal numbers of vertices. We observe that the
numbers of faces of each dimension must be equal to the dimensions of certain
representations of the automorphism groups of the corresponding Severi
varieties. We construct a complex involving these representations, which should
be considered as a geometric version of the (putative) triangulations
Observation of confined current ribbon in JET plasmas
we report the identification of a localised current structure inside the JET
plasma. It is a field aligned closed helical ribbon, carrying current in the
same direction as the background current profile (co-current), rotating
toroidally with the ion velocity (co-rotating). It appears to be located at a
flat spot in the plasma pressure profile, at the top of the pedestal. The
structure appears spontaneously in low density, high rotation plasmas, and can
last up to 1.4 s, a time comparable to a local resistive time. It considerably
delays the appearance of the first ELM.Comment: 10 pages, 6 figure
Characterisation of local ICRF heat loads on the JET ILW
When using Ion Cyclotron Range of Frequency (ICRF) heating, enhanced
heat-fluxes are commonly observed on some plasma facing components close to the
antennas. Experiments have recently been carried out on JET with the new
ITER-Like-Wall (ILW) to characterize the heat flux to the JET ICRF antennas.
Using Infra-Red thermography and thermal models of the tiles, heat-fluxes were
evaluated from the surface temperature increase during the RF phase of L-mode
plasmas. The maximum observed heat-flux intensity was ~ 4.5 MW/m2 when
operating with -{\pi}/2 current drive strap phasing at power level of 2MW per
antenna and with a 4 cm distance between the plasma and the outer limiters.
Heat-fluxes are reduced when using dipole strap phasing. The fraction of ICRF
power deposited on the antenna limiters or septa was in the range 2-10% for
dipole phasing and 10-20% with +/-{\pi}/2 phasing.Comment: 22 pages, 6 figure
Evaluation of X chromosome inactivation with respect to HLA genetic susceptibility in rheumatoid arthritis and systemic sclerosis
Background: Autoimmune diseases, including rheumatoid arthritis (RA) and systemic sclerosis (SSc) are characterized by a strong genetic susceptibility from the Human Leucocyte Antigen (HLA) locus. Additionally, disorders of epigenetic processes, in particular non-random X chromosome inactivation (XCI), have been reported in many female-predominant autoimmune diseases. Here we test the hypothesis that women with RA or SSc who are strongly genetically predisposed are less susceptible to XCI bias. Methods: Using methylation sensitive genotyping of the androgen receptor (AR) gene, XCI profiles were performed in peripheral blood mononuclear cells from 161 women with RA, 96 women with SSc and 100 healthy women. HLA-DRB1 and DQB1 were genotyped. Presence of specific autoantibodies was documented for patients. XCI skewing was defined as having a ratio ≥ 80:20 of cells inactivating the same X chromosome. Results: 110 women with RA, 68 women with SSc, and 69 controls were informative for the AR polymorphism. Among them 40.9% of RA patients and 36.8% of SSc patients had skewed XCI compared to 17.4% of healthy women (P = 0.002 and 0.018, respectively). Presence of RA-susceptibility alleles coding for the "shared epitope" correlated with higher skewing among RA patients (P = 0.002) and such correlation was not observed in other women, healthy or with SSc. Presence of SSc-susceptibility alleles did not correlate with XCI patterns among SSc patients. Conclusion: Data demonstrate XCI skewing in both RA and SSc compared to healthy women. Unexpectedly, skewed XCI occurs more often in women with RA carrying the shared epitope, which usually reflects severe disease. This reinforces the view that loss of mosaicism in peripheral blood may be a consequence of chronic autoimmunity. © 2016 Kanaan et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited
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