| 1. |
- Klionsky, Daniel J., et al.
(författare)
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Guidelines for the use and interpretation of assays for monitoring autophagy
- 2012
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Ingår i: Autophagy. - : Informa UK Limited. - 1554-8635 .- 1554-8627. ; 8:4, s. 445-544
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Forskningsöversikt (refereegranskat)abstract
- In 2008 we published the first set of guidelines for standardizing research in autophagy. Since then, research on this topic has continued to accelerate, and many new scientists have entered the field. Our knowledge base and relevant new technologies have also been expanding. Accordingly, it is important to update these guidelines for monitoring autophagy in different organisms. Various reviews have described the range of assays that have been used for this purpose. Nevertheless, there continues to be confusion regarding acceptable methods to measure autophagy, especially in multicellular eukaryotes. A key point that needs to be emphasized is that there is a difference between measurements that monitor the numbers or volume of autophagic elements (e.g., autophagosomes or autolysosomes) at any stage of the autophagic process vs. those that measure flux through the autophagy pathway (i.e., the complete process); thus, a block in macroautophagy that results in autophagosome accumulation needs to be differentiated from stimuli that result in increased autophagic activity, defined as increased autophagy induction coupled with increased delivery to, and degradation within, lysosomes (in most higher eukaryotes and some protists such as Dictyostelium) or the vacuole (in plants and fungi). In other words, it is especially important that investigators new to the field understand that the appearance of more autophagosomes does not necessarily equate with more autophagy. In fact, in many cases, autophagosomes accumulate because of a block in trafficking to lysosomes without a concomitant change in autophagosome biogenesis, whereas an increase in autolysosomes may reflect a reduction in degradative activity. Here, we present a set of guidelines for the selection and interpretation of methods for use by investigators who aim to examine macroautophagy and related processes, as well as for reviewers who need to provide realistic and reasonable critiques of papers that are focused on these processes. These guidelines are not meant to be a formulaic set of rules, because the appropriate assays depend in part on the question being asked and the system being used. In addition, we emphasize that no individual assay is guaranteed to be the most appropriate one in every situation, and we strongly recommend the use of multiple assays to monitor autophagy. In these guidelines, we consider these various methods of assessing autophagy and what information can, or cannot, be obtained from them. Finally, by discussing the merits and limits of particular autophagy assays, we hope to encourage technical innovation in the field.
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| 2. |
- Akiyama, Eiji, et al.
(författare)
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SPIRAL STRUCTURE AND DIFFERENTIAL DUST SIZE DISTRIBUTION IN THE LkH alpha 330 DISK
- 2016
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Ingår i: Astronomical Journal. - : American Astronomical Society. - 0004-6256 .- 1538-3881. ; 152:6
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Tidskriftsartikel (refereegranskat)abstract
- Dust trapping accelerates the coagulation of dust particles, and, thus, it represents an initial step toward the formation of planetesimals. We report H-band (1.6 mu m) linear polarimetric observations and 0.87 mm interferometric continuum observations toward a transitional disk around LkH alpha 330. As a. result, a pair of spiral arms were detected in the H-band emission, and an asymmetric (potentially arm-like) structure was detected in the 0.87 mm continuum emission. We discuss the origin of the spiral arm and the asymmetric structure. and suggest that a massive unseen planet is the most plausible explanation. The possibility of dust trapping and grain growth causing the asymmetric structure was also investigated through the opacity index (beta) by plotting the observed spectral energy distribution slope between 0.87 mm from our Submillimeter Array observation and 1.3 mm from literature. The results imply that grains are indistinguishable from interstellar medium-like dust in the east side (beta = 2.0 +/- 0.5) but are much smaller in the west side beta = 0.7(-0.4)(+0.5), indicating differential dust size distribution between the two sides of the disk. Combining the results of near-infrared and submillimeter observations, we conjecture that the spiral arms exist at the upper surface and an asymmetric structure resides in the disk interior. Future observations at centimeter wavelengths and differential polarization imaging in other bands (Y-K) with extreme AO imagers are required to understand how large dust grains form and to further explore the dust distribution in the disk.
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| 3. |
- Bakx, Tom J. L. C., et al.
(författare)
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ALMA uncovers the [C II] emission and warm dust continuum in a z=8.31 Lyman break galaxy
- 2020
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Ingår i: Monthly notices of the Royal Astronomical Society. - : Oxford University Press (OUP). - 0035-8711 .- 1365-2966. ; 493:3, s. 4294-4307
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Tidskriftsartikel (refereegranskat)abstract
- We report on the detection of the [C II] 157.7 mu m emission from the Lyman break galaxy (LBG) MACS0416_Y1 at z = 8.3113, by using the Atacama Large Millimeter/submillimeter Array (ALMA). The luminosity ratio of [O III] 88 mu m (from previous campaigns) to [CII] is 9.3 +/- 2.6, indicative of hard interstellar radiation fields and/or a low covering fraction of photodissociation regions. The emission of [C II] is cospatial to the 850 mu m dust emission (90 mu m rest frame, from previous campaigns), however the peak [C II] emission does not agree with the peak [O III] emission, suggesting that the lines originate from different conditions in the interstellar medium. We fail to detect continuum emission at 1.5 mm (160 mu m rest frame) down to 18 mu Jy (3 sigma). This non-detection places a strong limits on the dust spectrum, considering the 137 +/- 26 mu Jy continuum emission at 850 mu m. This suggests an unusually warm dust component (T > 80 K, 90 per cent confidence limit), and/or a steep dust-emissivity index (beta(dust) > 2), compared to galaxy-wide dust emission found at lower redshifts (typically T similar to 30-50 K, beta(dust) similar to 1-2). If such temperatures are common, thiswould reduce the required dust mass and relax the dust production problem at the highest redshifts. We therefore warn against the use of only single-wavelength information to derive physical properties, recommend a more thorough examination of dust temperatures in the early Universe, and stress the need for instrumentation that probes the peak of warm dust in the Epoch of Reionization.
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| 4. |
- Binggeli, Christian, et al.
(författare)
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A puzzling non-detection of [O III] and [C II] from a z ≈ 7.7 galaxy observed with ALMA
- 2021
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Ingår i: Astronomy and Astrophysics. - : EDP Sciences. - 0004-6361 .- 1432-0746. ; 646
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Tidskriftsartikel (refereegranskat)abstract
- Context. Characterizing the galaxy population in the early Universe holds the key to understanding the evolution of these objects and the role they played in cosmic reionization. However, there have been very few observations at the very highest redshifts to date. Aims. In order to shed light on the properties of galaxies in the high-redshift Universe and their interstellar media, we observe the Lyman-α emitting galaxy z7_GSD_3811 at z = 7.664 with bands 6 and 8 at the Atacama Large Millimeter/submillimeter Array (ALMA). Methods. We target the far-infrared [O iii] 88 µm and [C ii] 158 µm emission lines and dust continuum in the star-forming galaxy z7_GSD_3811 with ALMA. We combine these measurements with earlier observations in the rest-frame ultraviolet (UV) in order to characterize the object and compare the results to those of earlier studies that observed [O iii] and [C ii] emission in high-redshift galaxies. Results. The [O iii] 88 µm and [C ii] 158 µm emission lines are undetected at the position of z7_GSD_3811, with 3σ upper limits of 1.6 × 108 L and 4.0 × 107 L, respectively. We do not detect any dust continuum in band 6 nor band 8. The measured rms in the band 8 and band 6 continua are 26 and 9.9 µJy beam−1, respectively. Similar to several other high-redshift galaxies, z7_GSD_3811 exhibits low [C ii] emission for its star formation rate compared to local galaxies. Furthermore, our upper limit on the [O iii] line luminosity is lower than the previously observed [O iii] lines in high-redshift galaxies with similar UV luminosities. Our ALMA band 6 and 8 dust continuum observations imply that z7_GSD_3811 likely has a low dust content, and our non-detections of the [O iii] and [C ii] lines could indicate that z7_GSD_3811 has a low metallicity (Z. 0.1 Z).
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| 5. |
- Hashimoto, Motomu, et al.
(författare)
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Complement drives Th17 cell differentiation and triggers autoimmune arthritis
- 2010
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Ingår i: Journal of Experimental Medicine. - : Rockefeller University Press. - 0022-1007 .- 1540-9538. ; 207:6, s. 1135-1143
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Tidskriftsartikel (refereegranskat)abstract
- Activation of serum complement triggers Th17 cell-dependent spontaneous autoimmune disease in an animal model. In genetically autoimmune-prone SKG mice, administration of mannan or beta-glucan, both of which activate serum complement, evoked Th17 cell-mediated chronic autoimmune arthritis. C5a, a chief component of complement activation produced via all three complement pathways (i.e., lectin, classical, and alternative), stimulated tissue-resident macrophages, but not dendritic cells, to produce inflammatory cytokines including IL-6, in synergy with Toll-like receptor signaling or, notably, granulocyte/macrophage colony-stimulating factor (GM-CSF). GM-CSF secreted by activated T cells indeed enhanced in vitro IL-6 production by C5a-stimulated macrophages. In vivo, C5a receptor (C5aR) deficiency in SKG mice inhibited the differentiation/expansion of Th17 cells after mannan or beta-glucan treatment, and consequently suppressed the development of arthritis. Transfer of SKG T cells induced Th17 cell differentiation/expansion and produced arthritis in C5aR-sufficient recombination activating gene (RAG)(-/-) mice but not in C5aR-deficient RAG(-/-) recipients. In vivo macrophage depletion also inhibited disease development in SKG mice. Collectively, the data suggest that complement activation by exogenous or endogenous stimulation can initiate Th17 cell differentiation and expansion in certain autoimmune diseases and presumably in microbial infections. Blockade of C5aR may thus be beneficial for controlling Th17-mediated inflammation and autoimmune disease.
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| 6. |
- Hashimoto, Takuya, et al.
(författare)
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Big Three Dragons: A z=7.15 Lyman-break galaxy detected in [Oiii] 88 mu m, [Cii] 158 mu m, and dust continuum with ALMA
- 2019
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Ingår i: Publication of the Astronomical Society of Japan. - : Oxford University Press (OUP). - 2053-051X .- 0004-6264. ; 71:4
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Tidskriftsartikel (refereegranskat)abstract
- We present new ALMA observations and physical properties of a Lyman break galaxy at z = 7.15. Our target, B14-65666, has a bright ultra-violet (UV) absolute magnitude, M-UV approximate to -22.4, and has been spectroscopically identified in Ly alpha with a small rest-frame equivalent width of approximate to 4 angstrom. A previous Hubble Space TElescope (HST) image has shown that the target is composed of two spatially separated clumps in the rest-frame UV. With ALMA, we have newly detected spatially resolved [Oiii] 88 mu m, [Cii] 158 mu m, and their underlying dust continuum emission. In the whole system of B14-65666, the [Oiii] and [Cii] lines have consistent redshifts of 7.1520 +/- 0.0003, and the [Oiii] luminosity, (34.4 +/- 4.1)x 10(8)L(circle dot), is about three times higher than the [Cii] luminosity, (11.0 +/- 1.4) x 10(8)L(circle dot). With our two continuum flux densities, the dust temperature is constrained to be T-d approximate to 50-60K under the assumption of a dust emissivity index of beta(d) = 2.0-1.5, leading to a large total infrared luminosity of L-TIR approximate to 1 x 10(12)L(circle dot). Owing to our high spatial resolution data, we show that the [Oiii] and [Cii] emission can be spatially decomposed into two clumps associated with the two rest-frame UV clumps whose spectra are kinematically separated by approximate to 200kms(-1). We also find these two clumps have comparable UV, infrared, [Oiii], and [Cii] luminosities. Based on these results, we argue that B14-65666 is a starburst galaxy induced by a major merger. The merger interpretation is also supported by the large specific star formation rate (defined as the star formation rate per unit stellar mass), sSFR Gyr(-1), inferred from our SED fitting. Probably, a strong UV radiation field caused by intense star formation contributes to its high dust temperature and the [Oiii]-to-[Cii] luminosity ratio.
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| 7. |
- Hashimoto, Takuya, et al.
(författare)
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The onset of star formation 250 million years after the Big Bang
- 2018
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Ingår i: Nature. - : NATURE PUBLISHING GROUP. - 0028-0836 .- 1476-4687. ; 557:7705, s. 392-395
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Tidskriftsartikel (refereegranskat)abstract
- A fundamental quest of modern astronomy is to locate the earliest galaxies and study how they influenced the intergalactic medium a few hundred million years after the Big Bang(1-3). The abundance of star-forming galaxies is known to decline(4,5) from redshifts of about 6 to 10, but a key question is the extent of star formation at even earlier times, corresponding to the period when the first galaxies might have emerged. Here we report spectroscopic observations of MACS1149-JD1(6), a gravitationally lensed galaxy observed when the Universe was less than four per cent of its present age. We detect an emission line of doubly ionized oxygen at a redshift of 9.1096 +/- 0.0006, with an uncertainty of one standard deviation. This precisely determined redshift indicates that the red rest-frame optical colour arises from a dominant stellar component that formed about 250 million years after the Big Bang, corresponding to a redshift of about 15. Our results indicate that it may be possible to detect such early episodes of star formation in similar galaxies with future telescopes.
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| 8. |
- Kim, Hyeong Seok, et al.
(författare)
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Development, validation, and comparison of a nomogram based on radiologic findings for predicting malignancy in intraductal papillary mucinous neoplasms of the pancreas : An international multicenter study
- 2021
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Ingår i: Journal of hepato-biliary-pancreatic sciences. - : Wiley-Blackwell. - 1868-6974 .- 1868-6982.
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Although we previously proposed a nomogram to predict malignancy in intraductal papillary mucinous neoplasms (IPMN) and validated it in an external cohort, its application is challenging without data on tumor markers. Moreover, existing nomograms have not been compared. This study aimed to develop a nomogram based on radiologic findings and to compare its performance with previously proposed American and Korean/Japanese nomograms.METHODS: We recruited 3708 patients who underwent surgical resection at 31 tertiary institutions in eight countries, and patients with main pancreatic duct >10 mm were excluded. To construct the nomogram, 2606 patients were randomly allocated 1:1 into training and internal validation sets, and area under the receiver operating characteristics curve (AUC) was calculated using 10-fold cross validation by exhaustive search. This nomogram was then validated and compared to the American and Korean/Japanese nomograms using 1102 patients.RESULTS: Among the 2606 patients, 90 had main-duct type, 900 had branch-duct type, and 1616 had mixed-type IPMN. Pathologic results revealed 1628 low-grade dysplasia, 476 high-grade dysplasia, and 502 invasive carcinoma. Location, cyst size, duct dilatation, and mural nodule were selected to construct the nomogram. AUC of this nomogram was higher than the American nomogram (0.691 vs 0.664, P = .014) and comparable with the Korean/Japanese nomogram (0.659 vs 0.653, P = .255).CONCLUSIONS: A novel nomogram based on radiologic findings of IPMN is competitive for predicting risk of malignancy. This nomogram would be clinically helpful in circumstances where tumor markers are not available. The nomogram is freely available at http://statgen.snu.ac.kr/software/nomogramIPMN.
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| 9. |
- Kooistra, Robin, et al.
(författare)
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Radial decoupling of small and large dust grains in the transitional disk RX J1615.3-3255
- 2017
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Ingår i: Astronomy and Astrophysics. - : EDP Sciences. - 0004-6361 .- 1432-0746. ; 597
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Tidskriftsartikel (refereegranskat)abstract
- We present H-band (1.6 mu m) scattered light observations of the transitional disk RX J1615.3-3255, located in the similar to 1 Myr old Lupus association. From a polarized intensity image, taken with the HiCIAO instrument of the Subaru Telescope, we deduce the position angle and the inclination angle of the disk. The disk is found to extend out to 68 +/- 12 AU in scattered light and no clear structure is observed. Our inner working angle of 24 AU does not allow us to detect a central decrease in intensity similar to that seen at 30 AU in the 880 mu m continuum observations. We compare the observations with multiple disk models based on the spectral energy distribution (SED) and submm interferometry and find that an inner rim of the outer disk at 30 AU containing small silicate grains produces a polarized intensity signal which is an order of magnitude larger than observed. We show that a model in which the small dust grains extend smoothly into the cavity found for large grains is closer to the actual H-band observations. A comparison of models with di ff erent dust size distributions suggests that the dust in the disk might have undergone significant processing compared to the interstellar medium.
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| 10. |
- Landegren, Nils, et al.
(författare)
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A gene-centric approach to biomarker discovery identifies transglutaminase 1 as an epidermal autoantigen
- 2021
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Ingår i: Proceedings of the National Academy of Sciences of the United States of America. - : Proceedings of the National Academy of Sciences. - 0027-8424 .- 1091-6490. ; 118:51
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Tidskriftsartikel (refereegranskat)abstract
- Autoantigen discovery is a critical challenge for the understanding and diagnosis of autoimmune diseases. While autoantibody markers in current clinical use have been identified through studies focused on individual disorders, we postulated that a reverse approach starting with a putative autoantigen to explore multiple disorders might hold promise. We here targeted the epidermal protein transglutaminase 1 (TGM1) as a member of a protein family prone to autoimmune attack. By screening sera from patients with various acquired skin disorders, we identified seropositive subjects with the blistering mucocutaneous disease paraneoplastic pemphigus. Validation in further subjects confirmed TGM1 autoantibodies as a 55% sensitive and 100% specific marker for paraneoplastic pemphigus. This gene-centric approach leverages the wealth of data available for human genes and may prove generally applicable for biomarker discovery in autoimmune diseases.
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| 11. |
- Maruyama, Toshi, et al.
(författare)
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Hypothermia-induced tyrosine phosphorylation of SIRPα in the brain
- 2012
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Ingår i: Journal of Neurochemistry. - : John Wiley & Sons. - 0022-3042 .- 1471-4159. ; 121:6, s. 891-902
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Tidskriftsartikel (refereegranskat)abstract
- Signal regulatory protein α (SIRPα) is a neuronal membrane protein that undergoes tyrosine phosphorylation in the brain of mice in response to forced swim (FS) stress in cold water, and this response is implicated in regulation of depression-like behavior in the FS test. We now show that subjection of mice to the FS in warm (37°C) water does not induce the tyrosine phosphorylation of SIRPα in the brain. The rectal temperature (T(rec) ) of mice was reduced to 27° to 30°C by performance of the FS for 10 min in cold water, whereas it was not affected by the same treatment in warm water. The level of tyrosine phosphorylation of SIRPα in the brain was increased by administration of ethanol or picrotoxin, starvation, or cooling after anesthesia, all of which also induced hypothermia. Furthermore, the tyrosine phosphorylation of SIRPα in cultured hippocampal neurons was induced by lowering the temperature of the culture medium. CD47, a ligand of SIRPα, as well as Src family kinases or SH2 domain-containing protein phosphatase 2 (Shp2), might be important for the basal and the hypothermia-induced tyrosine phosphorylation of SIRPα. Hypothermia is therefore likely an important determinant of both the behavioral immobility and tyrosine phosphorylation of SIRPα observed in the FS test.
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| 12. |
- Mayama, Satoshi, et al.
(författare)
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Subaru Near-infrared Imaging Polarimetry of Misaligned Disks around the SR 24 Hierarchical Triple System
- 2020
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Ingår i: Astronomical Journal. - : American Astronomical Society. - 0004-6256 .- 1538-3881. ; 159:1
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Tidskriftsartikel (refereegranskat)abstract
- The SR 24 multistar system hosts both circumprimary and circumsecondary disks, which are strongly misaligned with each other. The circumsecondary disk is circumbinary in nature. Interestingly, both disks are interacting, and they possibly rotate in opposite directions. To investigate the nature of this unique twin disk system, we present 01 resolution near-infrared polarized intensity images of the circumstellar structures around SR 24, obtained with HiCIAO mounted on the Subaru 8.2 m telescope. Both the circumprimary disk and the circumsecondary disk are resolved and have elongated features. While the position angle of the major axis and radius of the near-IR (NIR) polarization disk around SR 24S are 55° and 137 au, respectively, those around SR 24N are 110° and 34 au, respectively. With regard to overall morphology, the circumprimary disk around SR 24S shows strong asymmetry, whereas the circumsecondary disk around SR 24N shows relatively strong symmetry. Our NIR observations confirm the previous claim that the circumprimary and circumsecondary disks are misaligned from each other. Both the circumprimary and circumsecondary disks show similar structures in 12CO observations in terms of its size and elongation direction. This consistency is because both NIR and 12CO are tracing surface layers of the flared disks. As the radius of the polarization disk around SR 24N is roughly consistent with the size of the outer Roche lobe, it is natural to interpret the polarization disk around SR 24N as a circumbinary disk surrounding the SR 24Nb–Nc system.
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| 13. |
- Nakamae, Toshio, et al.
(författare)
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Bone Marrow Edema and Low Back Pain in Elderly Degenerative Lumbar Scoliosis: A Cross-Sectional Study.
- 2016
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Ingår i: Spine. - 1528-1159. ; 41:10, s. 885-92
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Tidskriftsartikel (refereegranskat)abstract
- Cross-sectional study.To examine whether bone marrow edema is associated with low back pain in elderly patients with degenerative lumbar scoliosis.The cause of low back pain in degenerative lumbar scoliosis is unclear.A total of 120 degenerative lumbar scoliosis patients 65 years of age or older were evaluated. Radiography, computed tomography (CT), magnetic resonance imaging (MRI), and tender point examination in the lumbar spine were performed. On MRI, coronal gadolinium-contrasted T1- or T2-weighed fat-saturated images were used to score the size of bone marrow edema. The prevalence of bone marrow edema in patients with and without low back pain was compared; in patients with low back pain, we tested whether the locations of lumbar tender point were consistent with that of bone marrow edema.Bone marrow edema was found in 62 of 64 (96.9%) patients with low back pain compared with 21 of 56 (37.5%) patients without it (P<0.001). Bone marrow edema located more frequently on the concave side than on the convex side of scoliosis (P<0.001). Among patients with low back pain, bone marrow edema score was associated with low back pain severity (r=0.724; P<0.001), and the location of lumbar tender point were consistent with that of bone marrow edema (κ value=0.745; P<0.001).Bone marrow edema on MRI was closely associated with the presence of low back pain in elderly degenerative lumbar scoliosis.4.
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| 14. |
- Nakamae, Toshio, et al.
(författare)
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Efficacy of Percutaneous Vertebroplasty in the Treatment of Osteoporotic Vertebral Compression Fractures with Intravertebral Cleft.
- 2015
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Ingår i: The open orthopaedics journal. - : Bentham Science Publishers Ltd.. - 1874-3250. ; 9, s. 107-13
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Tidskriftsartikel (refereegranskat)abstract
- Intravertebral cleft (IVC) is frequently observed in patients with painful osteoporotic vertebral compression fracture (OVCF). Some studies reported the usefulness of percutaneous vertebroplasty (PVP) for treating OVCF with IVC. However, systematic studies are scarce, and their results are conflicting. The purpose of this study was to evaluate the clinical and radiographic results of PVP in the treatment of painful OVCF with IVC. Two hundred ninety-one patients with OVCF with IVC underwent PVP. Back pain was measured using a visual analog scale (VAS), and physical disability was assessed using the Oswestry Disability Index (ODI). Three radiological parameters were assessed: the local kyphotic angle, percentage spinal canal cross-sectional area of compromise, and intravertebral instability of the affected vertebra. The mean follow-up period was 28 months. The mean values for the VAS and ODI were 8.4 and 60.0%, respectively, before PVP, versus 3.9 and 35.4%, respectively, at the final follow-up. The average local kyphotic angle, percentage spinal canal cross-sectional area of compromise, and intravertebral instability were 10.5°, 17.9% and 6.1°, respectively, before PVP and 8.1°, 15.2%, and 0.8°, respectively, at the final follow-up. There were no neurological or systemic complications due to cement leakage. PVP is an effective and safe intervention for treating OVCF with IVC.
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| 15. |
- Oji, Vinzenz, et al.
(författare)
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Revised nomenclature and classification of inherited ichthyoses : Results of the First Ichthyosis Consensus Conference in Sorèze 2009
- 2010
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Ingår i: The Journal of American Academy of Dermatology. - : Elsevier BV. - 0190-9622 .- 1097-6787. ; 63:4, s. 607-641
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Inherited ichthyoses belong to a large, clinically and etiologically heterogeneous group of mendelian disorders of cornification, typically involving the entire integument. Over the recent years, much progress has been made defining their molecular causes. However, there is no internationally accepted classification and terminology. OBJECTIVE: We sought to establish a consensus for the nomenclature and classification of inherited ichthyoses. METHODS: The classification project started at the First World Conference on Ichthyosis in 2007. A large international network of expert clinicians, skin pathologists, and geneticists entertained an interactive dialogue over 2 years, eventually leading to the First Ichthyosis Consensus Conference held in Sorèze, France, on January 23 and 24, 2009, where subcommittees on different issues proposed terminology that was debated until consensus was reached. RESULTS: It was agreed that currently the nosology should remain clinically based. "Syndromic" versus "nonsyndromic" forms provide a useful major subdivision. Several clinical terms and controversial disease names have been redefined: eg, the group caused by keratin mutations is referred to by the umbrella term, "keratinopathic ichthyosis"-under which are included epidermolytic ichthyosis, superficial epidermolytic ichthyosis, and ichthyosis Curth-Macklin. "Autosomal recessive congenital ichthyosis" is proposed as an umbrella term for the harlequin ichthyosis, lamellar ichthyosis, and the congenital ichthyosiform erythroderma group. LIMITATIONS: As more becomes known about these diseases in the future, modifications will be needed. CONCLUSION: We have achieved an international consensus for the classification of inherited ichthyosis that should be useful for all clinicians and can serve as reference point for future research.
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| 16. |
- Saito, Yasuyuki, et al.
(författare)
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Regulation by SIRPα of dendritic cell homeostasis in lymphoid tissues
- 2010
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Ingår i: Blood. - : American Society of Hematology. - 0006-4971 .- 1528-0020. ; 116:18, s. 3517-3525
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Tidskriftsartikel (refereegranskat)abstract
- The molecular basis for regulation of dendritic cell (DC) development and homeostasis remains unclear. Signal regulatory protein α (SIRPα), an immunoglobulin superfamily protein that is predominantly expressed in DCs, mediates cell-cell signaling by interacting with CD47, another immunoglobulin superfamily protein. We now show that the number of CD11c(high) DCs (conventional DCs, or cDCs), in particular, that of CD8-CD4+ (CD4+) cDCs, is selectively reduced in secondary lymphoid tissues of mice expressing a mutant form of SIRPα that lacks the cytoplasmic region. We also found that SIRPα is required intrinsically within cDCs or DC precursors for the homeostasis of splenic CD4+ cDCs. Differentiation of bone marrow cells from SIRPα mutant mice into DCs induced by either macrophage-granulocyte colony-stimulating factor or Flt3 ligand in vitro was not impaired. Although the accumulation of the immediate precursors of cDCs in the spleen was also not impaired, the half-life of newly generated splenic CD4+ cDCs was markedly reduced in SIRPα mutant mice. Both hematopoietic and nonhematopoietic CD47 was found to be required for the homeostasis of CD4+ cDCs and CD8-CD4- (double negative) cDCs in the spleen. SIRPα as well as its ligand, CD47, are thus important for the homeostasis of CD4+ cDCs or double negative cDCs in lymphoid tissues.
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| 17. |
- Sato-Hashimoto, Miho, et al.
(författare)
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Microglial SIRP alpha regulates the emergence of CD11c(+) microglia and demyelination damage in white matter
- 2019
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Ingår i: eLIFE. - : eLIFE Sciences Publications. - 2050-084X. ; 8
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Tidskriftsartikel (refereegranskat)abstract
- A characteristic subset of microglia expressing CD11c appears in response to brain damage. However, the functional role of CD11c(+) microglia, as well as the mechanism of its induction, are poorly understood. Here we report that the genetic ablation of signal regulatory protein alpha (SIRP alpha), a membrane protein, induced the emergence of CD11c(+) microglia in the brain white matter. Mice lacking CD47, a physiological ligand of SIRP alpha, and microglia-specific SIRP alpha-knockout mice exhibited the same phenotype, suggesting that an interaction between microglial SIRP alpha and CD47 on neighbouring cells suppressed the emergence of CD11c(+) microglia. A lack of SIRP alpha did not cause detectable damage to the white matter, but resulted in the increased expression of genes whose expression is characteristic of the repair phase after demyelination. In addition, cuprizone-induced demyelination was alleviated by the microglia-specific ablation of SIRP alpha. Thus, microglial SIRP alpha suppresses the induction of CD11c(+) microglia that have the potential to accelerate the repair of damaged white matter.
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| 18. |
- Sato-Hashimoto, Miho, et al.
(författare)
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Signal regulatory protein α regulates the homeostasis of T lymphocytes in the spleen.
- 2011
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Ingår i: Journal of Immunology. - : American Association of Immunologists. - 0022-1767 .- 1550-6606. ; 187:1, s. 291-297
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Tidskriftsartikel (refereegranskat)abstract
- The molecular basis for formation of lymphoid follicle and its homeostasis in the secondary lymphoid organs remains unclear. Signal regulatory protein α (SIRPα), an Ig superfamily protein that is predominantly expressed in dendritic cells or macrophages, mediates cell-cell signaling by interacting with CD47, another Ig superfamily protein. In this study, we show that the size of the T cell zone as well as the number of CD4(+) T cells were markedly reduced in the spleen of mice bearing a mutant (MT) SIRPα that lacks the cytoplasmic region compared with those of wild-type mice. In addition, the expression of CCL19 and CCL21, as well as of IL-7, which are thought to be important for development or homeostasis of the T cell zone, was markedly decreased in the spleen of SIRPα MT mice. By the use of bone marrow chimera, we found that hematopoietic SIRPα is important for development of the T cell zone as well as the expression of CCL19 and CCL21 in the spleen. The expression of lymphotoxin and its receptor, lymphotoxin β receptor, as well as the in vivo response to lymphotoxin β receptor stimulation were also decreased in the spleen of SIRPα MT mice. CD47-deficient mice also manifested phenotypes similar to SIRPα MT mice. These data suggest that SIRPα as well as its ligand CD47 are thus essential for steady-state homeostasis of T cells in the spleen.
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| 19. |
- Tamura, Yoichi, et al.
(författare)
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Detection of the Far-infrared [O III] and Dust Emission in a Galaxy at Redshift 8.312 : Early Metal Enrichment in the Heart of the Reionization Era
- 2019
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Ingår i: Astrophysical Journal. - : IOP PUBLISHING LTD. - 0004-637X .- 1538-4357. ; 874:1
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Tidskriftsartikel (refereegranskat)abstract
- We present the Atacama Large Millimeter/submillimeter Array detection of the [O III] 88 mu m line and rest-frame 90 mu m dust continuum emission in a Y-dropout Lyman break galaxy (LBG), MACS0416_Y1 lying behind the Frontier Field cluster MACS J0416.1-2403. This [O III] detection confirms the LBG with a spectroscopic redshift of z = 8.3118 +/- 0.0003, making this object one of the farthest galaxies ever identified spectroscopically. The observed 850 mu m flux density of 137 +/- 26 mu Jy corresponds to a de-lensed total infrared (IR) luminosity of L-IR = (1.7 +/- 0.3) x 10(11) L-circle dot if assuming a dust temperature of T-dust = 50 K and an emissivity index of beta = 1.5, yielding a large dust mass of 4 x 10(6) M-circle dot. The ultraviolet-to-far-IR spectral energy distribution modeling where the [O III] emissivity model is incorporated suggests the presence of a young (tau(age) approximate to 4 Myr), star-forming (SFR approximate to 60 M-circle dot yr(-1)), moderately metal-polluted (Z approximate to 0.2 Z(circle dot)) stellar component with a mass of M-star = 3 x 10(8) M-circle dot. An analytic dust mass evolution model with a single episode of star formation does not reproduce the metallicity and dust mass in tau(age) approximate to 4 Myr, suggesting a pre-existing evolved stellar component with M-star similar to 3 x 10(9) M-circle dot and tau(age) similar to 0.3 Gyr as the origin of the dust mass.
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| 20. |
- Tamura, Yoichi, et al.
(författare)
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The 300 pc Resolution Imaging of a z=8.31 Galaxy : Turbulent Ionized Gas and Potential Stellar Feedback 600 Million Years after the Big Bang
- 2023
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Ingår i: Astrophysical Journal. - : Institute of Physics (IOP). - 0004-637X .- 1538-4357. ; 952:1
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Tidskriftsartikel (refereegranskat)abstract
- We present the results of 300 pc resolution Atacama Large Millimeter/submillimeter Array imaging of the [O iii] 88 & mu;m line and dust continuum emission from a z = 8.312 Lyman-break galaxy MACS0416_Y1. The velocity-integrated [O iii] emission has three peaks that are likely associated with three young stellar clumps of MACS0416_Y1, while the channel map shows a complicated velocity structure with little indication of a global velocity gradient unlike what was found in [C ii] 158 & mu;m at a larger scale, suggesting random bulk motion of ionized gas clouds inside the galaxy. In contrast, dust emission appears as two individual clumps apparently separating or bridging the [O iii]/stellar clumps. The cross-correlation coefficient between dust and ultraviolet-related emission (i.e., [O iii] and ultraviolet continuum) is unity on a galactic scale, while it drops at <1 kpc, suggesting well-mixed geometry of multiphase interstellar media on subkiloparsec scales. If the cutoff scale characterizes different stages of star formation, the cutoff scale can be explained by gravitational instability of turbulent gas. We also report on a kiloparsec-scale off-center cavity embedded in the dust continuum image. This could be a superbubble producing galactic-scale outflows, since the energy injection from the 4 Myr starburst suggested by a spectral energy distribution analysis is large enough to push the surrounding media creating a kiloparsec-scale cavity.
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| 21. |
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