| 1. |
- García-García, Andrés, et al.
(författare)
-
Culturing patient-derived malignant hematopoietic stem cells in engineered and fully humanized 3D niches
- 2021
-
Ingår i: Proceedings of the National Academy of Sciences of the United States of America. - : Proceedings of the National Academy of Sciences. - 0027-8424. ; 118:40
-
Tidskriftsartikel (refereegranskat)abstract
- Human malignant hematopoietic stem and progenitor cells (HSPCs) reside in bone marrow (BM) niches, which remain challenging to explore due to limited in vivo accessibility and constraints with humanized animal models. Several in vitro systems have been established to culture patient-derived HSPCs in specific microenvironments, but they do not fully recapitulate the complex features of native bone marrow. Our group previously reported that human osteoblastic BM niches (O-N), engineered by culturing mesenchymal stromal cells within three-dimensional (3D) porous scaffolds under perfusion flow in a bioreactor system, are capable of maintaining, expanding, and functionally regulating healthy human cord blood-derived HSPCs. Here, we first demonstrate that this 3D O-N can sustain malignant CD34+ cells from acute myeloid leukemia (AML) and myeloproliferative neoplasm patients for up to 3 wk. Human malignant cells distributed in the bioreactor system mimicking the spatial distribution found in native BM tissue, where most HSPCs remain linked to the niches and mature cells are released to the circulation. Using human adipose tissue-derived stromal vascular fraction cells, we then generated a stromal-vascular niche and demonstrated that O-N and stromal-vascular niche differentially regulate leukemic UCSD-AML1 cell expansion, immunophenotype, and response to chemotherapy. The developed system offers a unique platform to investigate human leukemogenesis and response to drugs in customized environments, mimicking defined features of native hematopoietic niches and compatible with the establishment of personalized settings.
|
|
| 2. |
|
|
| 3. |
|
|
| 4. |
- Roth, Phiippe, et al.
(författare)
-
Rüdlingerite, Mn2+2V5+As5+O7·2H2O, a New Species Isostructural with Fianelite
- 2020
-
Ingår i: Minerals. - : MDPI AG. - 2075-163X. ; 10:11, s. 1-15
-
Tidskriftsartikel (refereegranskat)abstract
- The new mineral species rüdlingerite, ideally Mn2+2V5+As5+O7·2H2O, occurs in the Fianel mine, in Val Ferrera, Grisons, Switzerland, a small Alpine metamorphic Mn deposit. It is associated with ansermetite and Fe oxyhydroxide in thin fractures in Triassic dolomitic marbles. Rüdlingerite was also found in specimens recovered from the dump of the Valletta mine, Canosio, Cuneo, Piedmont, Italy, where it occurs together with massive braccoite and several other As- and V-rich phases in richly mineralized veins crossing the quartz-hematite ore. The new mineral displays at both localities yellow to orange, flattened elongated prismatic, euhedral crystals measuring up to 300 μm in length. Electron-microprobe analysis of rüdlingerite from Fianel gave (in wt%): MnO 36.84, FeO 0.06, As2O5, 25.32, V2O5 28.05, SiO2 0.13, H2Ocalc 9.51, total 99.91. On the basis of 9 O anions per formula unit, the chemical formula of rüdlingerite is Mn1.97(V5+1.17 As0.83Si0.01)Σ2.01O7·2H2O. The main diffraction lines are [dobs in Å (Iobs) hkl]: 3.048 (100) 022, 5.34 (80) 120, 2.730 (60) 231, 2.206 (60) 16-1, 7.28 (50) 020, 2.344 (50) 250, 6.88 (40) 110, and 2.452 (40) 320. Study of the crystal structure showcases a monoclinic unit cell, space group P21/n, with a = 7.8289(2) Å, b = 14.5673(4) Å, c = 6.7011(2) Å, β = 93.773(2)°, V = 762.58(4) Å3, Z = 4. The crystal structure has been solved and refined to R1 = 0.041 on the basis of 3784 reflections with Fo > 4σ(F). It shows Mn2+ hosted in chains of octahedra that are subparallel to [-101] and bound together by pairs of tetrahedra hosted by V5+ and As5+, building up a framework. Additional linkage is provided by hydrogen-bonding through H2O coordinating Mn2+ at the octahedra. One tetrahedrally coordinated site is dominated by V5+, T(1)(V0.88As0.12), corresponding to an observed site scattering of 24.20 electrons per site (eps), whereas the second site is strongly dominated by As5+, T(2)(As0.74V0.26), with, accordingly, a higher observed site scattering of 30.40 eps. The new mineral has been approved by the IMA-CNMNC and named for Gottfried Rüdlinger (born 1919), a pioneer in the 1960–1980s, in the search and study of the small minerals from the Alpine manganese mineral deposits of Grisons.
|
|
| 5. |
- Škoda, Radek, et al.
(författare)
-
Gadolinite-(Nd), a new member of the gadolinite supergroup from Fe-REE deposits of Bastnäs-type.
- 2018
-
Ingår i: Mineralogical magazine. - : Mineralogical Society. - 0026-461X .- 1471-8022. ; 82(S1), s. S133-S145
-
Tidskriftsartikel (refereegranskat)abstract
- A new member of the gadolinite supergroup, gadolinite-(Nd), IMA2016-013, ideally Nd2FeBe2Si2O10, was found in the Malmkärra mine, ~3.5 km WSW of Norberg, south-central Sweden, where it occurs in association with fluorbritholite-(Ce), västmanlandite-(Ce), dollaseite-(Ce), bastnäsite-(Ce) and tremolite. Gadolinite-(Nd) forms anhedral grains up to 150 µm in size, commonly occurring as aggregates of olive green colour. The crystals are transparent with vitreous to adamantine lustre. Gadolinite-(Nd) is brittle with conchoidal fracture, no cleavage or parting was observed. It has a white streak, the Mohs hardness is 6.5–7 and the calculated density is 4.86 g cm–3. Optically, the mineral is weakly pleochroic in shades of olive green, biaxial (–), nα = 1.78(1), nβ(calc.) = 1.80, nγ = 1.81(1) measured in white light, 2V(meas.) = 62(3)°. Electron-microprobe and laser ablation inductively coupled plasma mass spectrometry analysis [in wt.%] provided SiO2 21.77, Y2O3 5.49, La2O3 2.78, Ce2 O3 14.04, Pr2O3 3.28, Nd2O3 19.27, Sm2O3 5.30, Eu2O3 0.24, Gd2O3 4.10, Tb2O3 0.36, Dy2O3 1.32, Ho2O3 0.18, Er2O3 0.38, MgO 0.51, CaO 0.14, MnO 0.10, FeO 10.62, B2O3 0.10, BeOcalc. 8.99, H2Ocalc. 0.55 and total 99.52 giving the following empirical formula (based on 2 Si): (Nd0.632 Ce0.472Y0.268Sm0.168Gd0.125Pr0.110La0.094Dy0.039Ca0.014Er0.011Tb0.011Eu0.008Ho0.008)∑1.957(Fe0.816Mg0.070Mn0.008)∑0.894(Be1.984B0.016)∑2.000Si2O9.660OH0.337. A weak Raman vibration band at ~3525 cm–1 confirms the presence of water in the structure. Gadolinite-(Nd) is monoclinic, P21/c, with a = 4.8216(3) Å, b = 7.6985(4) Å, c = 10.1362(6) Å, β = 90.234(4)°, V = 376.24(6) Å3 and Z = 2. The strongest X-ray diffraction lines are [dobs in Å (hkl) Irel]: 4.830 (100) 72, 3.603 (021) 37, 3.191(-112) 52, 3.097 (013) 35, 2.888 (121) 100, 2.607(113) 49, 2.412 (200) 24. Along with the Malmkärra mine, gadolinite-(Nd) was also recorded also at Johannagruvan and Nya Bastnäs. The minerals of the gadolinite subgroup together with fluorbritholite-(Ce) incorporate the highest fraction of medium-to-heavy rare-earth elements among associated rare-earth element minerals in the Malmkärra mine and possibly in all Bastnäs-type deposits.
|
|
| 6. |
- Skoda, Radek, et al.
(författare)
-
Redefinition of thalenite-(Y) and discreditation of fluorthalenite-(Y) : A re-investigation of type material from the Osterby pegmatite, Dalarna, Sweden, and from additional localities
- 2015
-
Ingår i: Mineralogical magazine. - : Mineralogical Society. - 0026-461X .- 1471-8022. ; 79:4, s. 965-983
-
Tidskriftsartikel (refereegranskat)abstract
- Using type material from the Osterby pegmatite in Dalarna, Sweden, the chemical composition and structural parameters of thalenite-(Y) [ideally Y3Si3O10(OH)] were examined by wavelength dispersive spectroscopy electron microprobe (WDS EMP) analysis and single-crystal X-ray diffraction. High contrast back-scatter electron images of the Osterby material show at least two generations of thalenite-(Y). The formula of the primary thalenite-(Y) normalized to 11 anions is (Y2.58Dy0.11Yb0.09Gd0.06Er0.06Ho0.02 Sm0.02Tb0.02Lu0.02Nd0.01Tm0.01)(Sigma 3.00)Si3.01O10F0.97OH0.03. The secondary thalenite-(Y), replacing the primary material, is weakly enhanced in Y and depleted in the lightest and the heaviest rare-earth elements, yielding the formula (Y2.63Dy0.12Yb0.06Gd0.06Er0.05Ho0.02Sm0.02Tb0.02Tm0.01Nd0.01Lu0.01)(Sigma 3.00) Si3.01O10F0.98OH0.02. Structural data for thalenite-(Y) from Osterby clearly indicate the monoclinic space group P2(1)/n, with a = 7.3464(4), b = 11.1726(5), c = 10.4180(5) angstrom, beta = 97.318(4)degrees, V = 848.13(7) angstrom(3), Z = 4, which is consistent with previous investigations. The structure was refined from single-crystal X-ray diffraction data to R-1 = 0.0371 for 1503 unique observed reflections, and the final chemical composition obtained from the refinement, (Y2.64Dy0.36)(Sigma 3.00)F-0.987[Si3O10], Z = 4, is in good agreement with the empirical formula resulting from electron microprobe (EMP) analysis. Both techniques reveal a strong dominance of F over OH, which means that the type material actually corresponds to the fluorine analogue. Moreover, new EMP analyses of samples of thalenite-(Y) from an additional seven localities (angstrom skagen and Reunavare in Sweden; White Cloud and Snow Flake in Colorado, USA; the Guy Hazel claim in Arizona, USA; Suishoyama and Souri in Japan) clearly show the prevalence of F over OH as well. Based on these observations, the Commission on New Minerals, Nomenclature and Classification of the International Mineralogical Association has recommended a redefinition of the chemical composition of thalenite-(Y) to represent the F-dominant species with the ideal formula Y3Si3O10F, as it has historical priority. Consequently, the later described fluorthalenite-(Y) has to be discredited.
|
|
| 7. |
- Sobas, Marta, et al.
(författare)
-
Real-world study of children and young adults with myeloproliferative neoplasms: identifying risks and unmet needs
- 2022
-
Ingår i: BLOOD ADVANCES. - : ELSEVIER. - 2473-9529 .- 2473-9537. ; 6:17, s. 5171-5183
-
Tidskriftsartikel (refereegranskat)abstract
- Myeloproliferative neoplasms (MPNs) are uncommon in children/young adults. Here, we present data on unselected patients diagnosed before 25 years of age included from 38 centers in 15 countries. Sequential patients were included. We identified 444 patients, with median follow-up 9.7 years (0-47.8). Forty-nine (11.1%) had a history of thrombosis at diagnosis, 49 new thrombotic events were recorded (1.16% patient per year [pt/y]), perihepatic vein thromboses were most frequent (47.6% venous events), and logistic regression identified JAK2V617F mutation (P = .016) and hyperviscosity symptoms (visual disturbances, dizziness, vertigo, headache) as risk factors (P = .040). New hemorrhagic events occurred in 44 patients (9.9%, 1.04% pt/y). Disease transformation occurred in 48 patients (10.9%, 1.13% pt/y), usually to myelofibrosis (7.5%) with splenomegaly as a novel risk factor for transformation in essential thrombocythemia (ET) (P= .000) in logistical regression. Eight deaths (1.8%) were recorded, 3 after allogeneic stem cell transplantation. Concerning conventional risk scores: International Prognostic Score for Essential Thrombocythemia-Thrombosis and new International Prognostic Score for Essential Thrombocythemia-Thrombosis differentiated ET patients in terms of thrombotic risk. Both scores identified high-risk patients with the same median thrombosis-free survival of 28.5 years. No contemporary scores were able to predict survival for young ET or polycythemia vera patients. Our data represents the largest real-world study of MPN patients age < 25 years at diagnosis. Rates of thrombotic events and transformation were higher than expected compared with the previous literature. Our study provides new and reliable information as a basis for prospective studies, trials, and development of harmonized international guidelines for the specific management of young patients with MPN.
|
|
