Niemann Pick disease: a rare lysosomal storage disease

Niemann Pick Disease (NPD) is a rare autosomal recessive lysosomal storage disease characterized by lysosomal lipid storage. The disease is caused by deficiency of enzyme, acid sphingomyelinase (ASM) which leads to accumulation of sphingomyelin & other lipids in reticuloendothelial cells of various organs like liver, spleen, bone marrow, lymph node, brain, nerves and kidney. Four types of the disease have been identified i.e. A, B, C and D. We report a case of Niemann Pick Disease type C. The patient was a 2.5 years female child who presented with developmental regression, recurrent seizures, failure to thrive and hepatospleenomegaly. Bone marrow (BM) aspiration was performed which showed hypercelluler marrow with few fat laden macrophage resembling foam cell that are characteristics of this disease. BSMMU J 2022; 15(2): 141-14

UPAYA MENINGKATKAN MOTIVASI BELAJAR SISWA MELALUI MEDIA GAME EDUCATION DALAM PEMBELAJARAN IPS DI MTs HASBUR RACHMAN ALIF DESA LENGKONG WETAN KABUPATEN MAJALENGKA

ASEP SAMSUL MA'ARIF : Berdasarkan Observasiawal di kelas VII MTs Hasbur Rachman Alif terlihat proses pembelajaran IPS masih menggunakan media spidol dan papan tulis. Sehingga banyak siswa yang kurang memperhatikan guru pada saat proses pembelajaran., Hal ini berakibat pada kejenuhan dan kebosanan yang berdampak pada hasil belajar siswa yang kurang dari KKM yang diterapkan di MTs Hasbur Rachman Alif yakni 70. Tujuan penelitian ini untuk mengetahui bagaimana penerapan pembelajaran dengan menggunakan media Game Education pada mata pelajaran IPS Terpadu, aktivitas siswa dan kinerja guru dengan pembelajaran dengan menggunakan media Game Education, dan mengetahui motivasi belajar siswa sebelum dan setelah diterapkannya pembelajaran dengan menggunakan media Game education pada mata pelajaran IPS Terpadu di MTs Hasbur Rachman Alif. Pembelajaran dengan menggunakan media Game Education menekankan motivasi siswa untuk berpikir kritis dan menuangkan. Hal ini memudahkan siswa untuk mengakses materi IPS yang banyak dimanapun, serta mempemudah daya ingat siswa tentang apayatgtelah didapatkan selama proses pembelajaran. Penelitian ini menggunakan Penelitian Tindakan Kelas (pTK) yang dilaksanakan selama tiga siklus. Teknik Pengumpulan data menggunakan observasi, tes, dan dokumentasi. Subjek penelitian ini adalah siswa kelas VII MTs Hasbur Rachman Alif sebanyak 31 siswa yang terdiri dari 18 siswa laki-laki dan 13 siswa perempuan. Pembelajaran dengan menggunakan media Game Education dapat meningkatkan motivasi belajar siswa hal ini terlihat dari hasil tes yang telah dilakukan selama tiga siklus, dimana pada pratindakan (pretes) nilai sebesar 65,25 dan ketuntasan belajar 25 yo, siklus I nilai rata-rata sebesar 76,75 dan ketuntasan_ belajar siswa sebesar 65,5%o siklus II nilai rata-rata siswa 85,75 dan nilai ketuntasan belajar sebesar 90% siklus III dengan nilai rata-rata 92,25 dan ketuntasan belajar mengalami peningkatanya itu 97,5yo. Dengan demikian dapat disimpulkan bahwa pembelajaran dengan menggunakan media Game Education merupakan salah satu upaya untuk meningkatkan hasil belajar siswa pada mata pelajaran IPS Terpadu di MTs Hasbur Rachman Alif. Kata Kunci: Game Education, MotivasiBelajar, PTK

The Behavior, onḂ0,11, of an Oscillatory Integral with Polynomial Phase Function

AbstractWe study the convolution oscillatory singular integral operatorTf=p.v.Ω∗f, with Ω(x)=eiq(x)K(x), whereqis a real-valued polynomial of a real variable, of degreed≥2, andKis a Calderón–Zygmund-type kernel. We prove that this operator extends to an operator that maps the Besov spaceḂ0,11into the Hardy-type spaceH10

Dehydroepiandrosterone sulfate mediates activation of transcription factors CREB and ATF-1 via a Gα11-coupled receptor in the spermatogenic cell line GC-2

AbstractDehydroepiandrosterone sulfate (DHEAS) is a circulating steroid produced in the adrenal cortex, brain, and gonads. Whereas a series of investigations attest to neuroprotective effects of the steroid in the brain, surprisingly little is known about the physiological effects of DHEAS on cells of the reproductive system. Here we demonstrate that DHEAS acting on the spermatogenic cell line GC-2 induces a time- and concentration-dependent phosphorylation of c-Src and Erk1/2 and activates the transcription factors activating transforming factor-1 (ATF-1) and cyclic AMP-responsive element binding protein (CREB). These actions are consistent with the non-classical signaling pathway of testosterone and suggest that DHEAS is a pro-androgen that is converted into testosterone in order to exert its biological activity. The fact, however, that steroid sulfatase mRNA was not detected in the GC-2 cells and the clear demonstration of DHEAS-induced activation of Erk1/2, ATF-1 and CREB after silencing the androgen receptor by small interfering RNA (siRNA) clearly contradict this assumption and make it appear unlikely that DHEAS has to be converted in the cytosol into a different steroid in order to activate the kinases and transcription factors mentioned. Instead, it is likely that the DHEAS-induced signaling is mediated through the interaction of the steroid with a membrane-bound G-protein-coupled receptor, since silencing of Guanine nucleotide-binding protein subunit alpha-11 (Gnα11) leads to the abolition of the DHEAS-induced stimulation of Erk1/2, ATF-1, and CREB. The investigation presented here shows a hormone-like activity of DHEAS on a spermatogenic cell line. Since DHEAS is produced in male and female reproductive organs, these findings could help to define new roles for DHEAS in the physiology of reproduction

Measurement of charm and beauty production at central rapidity versus charged-particle multiplicity in proton-proton collisions at s\sqrt{s} = 7 TeV

Prompt D meson and non-prompt J/$\psi$ yields are studied as a function of the multiplicity of charged particles produced in inelastic proton-proton collisions at a centre-of-mass energy of $\sqrt{s}=7$ TeV. The results are reported as a ratio between yields in a given multiplicity interval normalised to the multiplicity-integrated ones (relative yields). They are shown as a function of the multiplicity of charged particles normalised to the average value for inelastic collisions (relative charged-particle multiplicity). D\)^0\), D\)^+\) and D\)^{*+}\) mesons are measured in five $p_{\rm T}$ intervals from 1 to 20 GeV/$c$ and for $|y|1.3$ GeV/$c$ and $|y|0$, The fraction of non-prompt J/$\psi$ in the inclusive J/$\psi$ yields shows no dependence on the charged-particle multiplicity at central rapidity. Charm and beauty hadron relative yields exhibit a similar increase with increasing charged-particle multiplicity. The measurements are compared to PYTHIA 8, EPOS 3 and percolation calculations.publishedVersio

Study of J/ψ azimuthal anisotropy at forward rapidity in Pb-Pb collisions at √sNN=5.02 TeV

The second (v2) and third (v3) flow harmonic coefficients of J/ψ mesons are measured at forward rapidity (2.5 < y < 4.0) in Pb-Pb collisions at √ sNN = 5.02 TeV with the ALICE detector at the LHC. Results are obtained with the scalar product method and reported as a function of transverse momentum, pT, for various collision centralities. A positive value of J/ψ v3 is observed with 3.7σ significance. The measurements, compared to those of prompt D0 mesons and charged particles at mid-rapidity, indicate an ordering with vn(J/ψ) < vn(D0 ) < vn(h±) (n = 2, 3) at low and intermediate pT up to 6 GeV/c and a convergence with v2(J/ψ) ≈ v2(D0 ) ≈ v2(h±) at high pT above 6–8 GeV/c. In semicentral collisions (5–40% and 10–50% centrality intervals) at intermediate pT between 2 and 6 GeV/c, the ratio v3/v2 of J/ψ mesons is found to be significantly lower (4.6σ) with respect to that of charged particles. In addition, the comparison to the prompt D0 -meson ratio in the same pT interval suggests an ordering similar to that of the v2 and v3 coefficients. The J/ψ v2 coefficient is further studied using the Event Shape Engineering technique. The obtained results are found to be compatible with the expected variations of the eccentricity of the initial-state geometry.publishedVersio

Measurement of isolated photon-hadron correlations in √sNN = 5.02 TeV pp and p-Pb collisions

This paper presents isolated photon-hadron correlations using pp and p-Pb data collected by the ALICE detector at the LHC. For photons with |η|<0.67 and 12<pT<40 GeV/c, the associated yield of charged particles in the range |η|<0.80 and 0.5<pT<10 GeV/c is presented. These momenta are much lower than previous measurements at the LHC. No significant difference between pp and p-Pb is observed, with Pythia 8.2 describing both data sets within uncertainties. This measurement constrains nuclear effects on the parton fragmentation in p-Pb collisions, and provides a benchmark for future studies of Pb-Pb collisions.publishedVersio

Constraining the Chiral Magnetic Effect with charge-dependent azimuthal correlations in Pb-Pb collisions at sNN\sqrt{s_{NN}} = 2.76 and 5.02 TeV

Systematic studies of charge-dependent two- and three-particle correlations in Pb-Pb collisions at $\sqrt{s_{NN}}$ = 2.76 and 5.02 TeV used to probe the Chiral Magnetic Effect (CME) are presented. These measurements are performed for charged particles in the pseudorapidity (η) and transverse momentum (pT) ranges |η| < 0.8 and 0.2 < pT < 5 GeV/c. A significant charge-dependent signal that becomes more pronounced for peripheral collisions is reported for the CME-sensitive correlators γ1, 1 = 〈cos(φα + φβ − 2Ψ2)〉 and γ1, − 3 = 〈cos(φα − 3φβ + 2Ψ2)〉. The results are used to estimate the contribution of background effects, associated with local charge conservation coupled to anisotropic flow modulations, to measurements of the CME. A blast-wave parametrisation that incorporates local charge conservation tuned to reproduce the centrality dependent background effects is not able to fully describe the measured γ1,1. Finally, the charge and centrality dependence of mixed-harmonics three-particle correlations, of the form γ1, 2 = 〈cos(φα + 2φβ − 3Ψ3)〉, which are insensitive to the CME signal, verify again that background contributions dominate the measurement of γ1,1.publishedVersio