bluuebunny/crossref_metadata_2025_split huggingface dataset:
Pharmacological chaperones glucocerebrosidase as treatment of Gaucher disease
Authors: Копытова А.Э. , Николаев М.А. , Богданова Д.А. , Сенкевич К.А. , Байдакова Г.В. , Большакова О.И. , Саранцева С.В. , Рычков Г.Н. , Чеблоков А.А. , Ибатуллин Ф.М. , Милюхина И.В. , Захарова Е.Ю. , Пчелина С.Н. , Емельянов А.К. 
Published: November 2020

Мутации в гене лизосомного фермента глюкоцереброзидазы (GBA), приводящие к дисфункции фермента глюкоцереброзидазы (GCase), в гомозиготном состоянии вызывают болезнь Гоше (БГ), а в гетерозиготном состоянии повышают риск развития болезни Паркинсона (БП). Обсуждается использование фармакологических шаперонов (ФШ) GСase для терапии как БГ, так и GBA-ассоциированной БП (GBA-БП). Используя ФШ амброксол мы показали увеличение ферментативной активности GCase и снижение концентрации лизосфинголипидов HexSph в культуре первичных макрофагов пациентов с БГ и GBA-БП. Впервые проведена оценка эффективности новых химических соединений, модификаций ранее описанного аллостерического ФШ.
Gaucher disease (GD), caused by GBA mutations, encodes lysosomal enzyme glucocerebrosidase (GCase). Pharmacological chaperones could potentially enhance GCase activity and treat GD and PD linked to mutations in the GBA gene (GBA-PD). Using ABX as pharmacological chaperone for macrophages derived from GD and GBA-PD patients we observed significantly enhanced GCase activity and decrease of HexSph concentration. For the first time was evaluated the effectiveness of chemical modifications of the previously described allosteric pharmacological chaperone.
Conflitos entre criadores da raça Moxotó e animais silvestres no Semiárido pernambucano
Authors: De Medeiros Rosália Barros Nascimento , Arandas Janaina Kelli Gomes , Alves Ângelo Giuseppe Chaves , Alves Rômulo Romeu Nóbrega , Ribeiro Maria Norma 
Published: November 2020

Este artigo tem como objetivos identificar conflitos existentes entre criadores de caprinos da raça Moxotó e os predadores naturais dos seus animais, no município de Ibimirim, Pernambuco, bem como descrever as formas locais de prevenção aos ataques e suas implicações para conservação de raças caprinas e espécies silvestres. As informações foram obtidas através de entrevistas com questionários semiestruturados, complementadas com listas-livres. Foram entrevistados nove criadores, considerados especialistas locais. Foram identificadas 13 espécies selvagens como as principais causadoras de danos para as criações de caprinos da região. As espécies citadas foram carcará (Caracara plancus), gato-mourisco (Puma yagouaroundi), conhecido e designado na região pelos criadores como gato-do-mato azul e vermelho, jiboia (Boa constrictor), urubu (Coragyps atratus), cão doméstico (Canis familiaris), raposa (Cerdocyon thous), cascavel (Crotalus durissus), morcego (Desmodus rotundus), jararaca (Bothropoides erythromelas), onça-parda (Puma concolor) e gavião (espécie não-identificada), em ordem decrescente de importância. O conflito entre criadores de caprinos e os animais silvestres se deve aos prejuízos econômicos causados pelos ataques. Os criadores diferenciam as espécies predadoras principalmente pela forma e intensidade dos ataques. Em geral, a caça ainda é a principal forma de prevenção dos ataques, porém, registraram-se formas locais de defesa para algumas espécies. É necessário que se estabeleça um diálogo constante entre os criadores e os órgãos ambientais responsáveis pelas políticas públicas com o objetivo de desenvolver estratégias para a conservação das raças locais, como a raça Moxotó, e espécies silvestres.
Ocorrência de Listeria spp. em salame tipo Italiano comercializados em supermercados do bairro de Casa Amarela, Recife-PE
Authors: Pessoa Camila Araújo Sousa de Sá , Aragão Breno Bezerra , Costa Caio Alves da , Santos Haliny Cristina Silva , Silva Maria Goretti Varejão da , Moura Andrea Paiva Botelho Lapenda de 
Published: November 2020

Atualmente, os alimentos prontos para consumo têm se popularizado. Isto se deve ao fato da conveniência e propriedades nutricionais encontradas nesses produtos. Embora sofram processamento tecnológico na fabricação, falhas higiênico-sanitárias podem se tornar um sério risco à saúde do consumidor devido à veiculação de patógenos. Dentre estes, destaca-se o gênero Listeria que tem importância para a indústria de alimentos e saúde pública. Assim, objetivou-se neste estudo a detecção de Listeria spp. em salames tipo Italiano fatiados, embalados a vácuo e comercializados em supermercados do bairro de Casa Amarela, Recife-PE. Foram coletadas 22 amostras de salames tipo Italiano fatiados, embalados a vácuo das três principais marcas brasileiras, estas foram analisadas de acordo com a metodologia da Instrução Normativa de nº62/2003 do Ministério da Agricultura Pecuária e Abastecimento. Dentre as amostras estudadas foi detectada uma ocorrência de 13,64% (3/22), este dado revela possíveis falhas na elaboração do alimento estudado, além de evidenciar a presença de Listeria spp. em salames tipo Italiano fatiados e embalados a vácuo.
Project Based Learning Model in Teaching Deutsch FÅ±r Tourismus for Foreign Language Students
Authors: Anwar Muhammad , Yusri Yusri , Angreany Femmy , Syaputra Arlian Fachrul , Hasmawati Hasmawati 
Published: November 2020

This study aims to determine the effectiveness of the application of PBL (Project Based Learning) learning models in Deutsch fÃ¼r Tourismus courses in foreign language majors. This research is an experimental study using one group design as an experimental group without involving the control group. Respondents in this study were 30 students who were programming the Deutsch fuer Tourismus course. Student speaking skills data were measured using assessment rubrics with intervals of 1 to 9. This assessment rubric was adapted from assessments in the IELTS test specifically in aspects of speaking skills. The intervention given was in the form of implementing the PBL model for 6 meetings, where each meeting was 2 hours long. Data analysis techniques used are descriptive statistics and inferential statistics. There are 4 types of advantages of the PBL model that are felt by students when implemented in the learning process namely 1) can trigger students to find and explore ideas or ideas that they want to express when speaking. 2) train students to work in groups to solve a problem. 3) This model produces products that are self-developed by students. 4) The PBL model requires students to read a lot, so that it can have a positive effect in increasing student reading interest.Â Keywords: Learning Model; Project Based Learning; Deutsch fÃ¼r Tourismus; Speaking Skills
UJI APLIKASI BERBAGAI TINGKAT DOSIS PUPUK ZA TERHADAP PRODUKTIVITAS DAN MUTU JAGUNG
Authors: Tabri Fahdiana , Aqil M. , Efendi Roy 
Published: November 2020

This study aims to see the effect of optimal dosage of ZA fertilizer on maize productivity and quality. The results showed that the treatment without urea, in the treatment of NPK 300 / kg + ZA 100 kg / ha fertilizer dosage will decrease the yield from 10.8 tons / ha to 9.1 tons / ha, and BC Ratio 2.34 to 2, 08. Further treatment without urea (NPK + Urea) has the highest production of NPK fertilizer 300 kg / ha + ZA 200 kg / ha with profit rate Rp. 20.435.000, - and B-C Ratio 2.21. Furthermore the addition of ZA fertilizer doses will actually reduce the production and B-C Ratio
Pengaruh Biaya Promosi terhadap Profitabilitas pada PT. Simpatik Cabang Gowa
Authors: Ardin Ardin 
Published: November 2020

The aims of the research are 1) To analyze the influence of cost promotion consisted of advertisement, sale promotion, Personal Sale and the publicity of the profitability of Simpatik in Gowa branch, and 2) To analyze the influence of the most dominant variable on the profitability of Simpatik in Gowa branch. The research was conducted by the same of population and sample taken from three years lately included thirty-six months by financing corporation data in Gowa branch. The method used was analyzes of classic method and doubled linear regression by using SPSS. 20. The result research through simultaneous testing showed that advertisement, sale promotion, personal sale and publicity variable gave the positive and significant influenced toward the profitability. Testing by partial way, the promotion and sale personality variable created negative influence and gave return equivalent toward the corporation of sale of Simpatik in Gowa branch. Next, in partial way, advertisement variable showed the most dominant influence toward profitability on the three of the others independent variable.
Pendekatan Perspektif Weber terhadap Tindakan Rasionalisme Pembuatan Perahu Pinisi
Authors: Dwi Hastuti Diah Retno , Mardia Mardia , Nuryanti Dewi Marwati , Ali Muhammad Saleh , Demmalino Eymal B. , Rahmadanih Rahmadanih 
Published: November 2020

Indonesia's maritime triumph from history to being written in a song lyric is not a question, especially its reliability in making Pinisi boats whose processes are full of local wisdom and cultural values reflected in everyday life. The research conducted in Bira Village, Bontobahari Sub-district, Bulukumba District in May 2018 aims to examine the rationalism of making pinisi boats with Weber's perspective approach. The aim is to use qualitative methods with Weber's approach in the verstehen perspective. The findings show that the making of the Pinisi boat is still filled with mystical nuances of religion ranging from the determination of the day of manufacture to its launch in the sea even though the elements of modernization began to come in to streamline time and streamline costs as a form of rationality in acting, as in Weber's perspective, zwerk rational, werk rational, affectual action, and traditional actions
Pengaruh Penerapan Studi Kasus terhadap Pengetahuan Pelanggaran Kode Etik Profesi Bidang IT bagi Mahasiswa Otomasi Sistem Permesinan
Authors: Rahmawati Nur Asmi 
Published: November 2020

This study was conducted to determine the effect of the application of case studies on knowledge of violations of the professional code of ethics in the IT field for students majoring in Mechanical Systems Automation. The research method used was an experiment with the design of "one shot case study". The study was conducted on students who program Professional Ethics courses majoring in Mechanical Systems Automation. The results of this study indicate that the value of students after doing a case study has increased. This shows that the case study method in this learning is appropriate to use.
The Implication of Sustainable Career Development Implementation (PKB) On Teachers’ Career Management in an Education Unit
Authors: Nurhayati Yuli , Permana Johar , Herawan Endang , Aedi Nur 
Published: November 2020

This study aims to examine the implications of the implementation of the Pengembanagan Keprofesian Berkelanjutan (PKB) policy on teacher career management in junior high schools in Bandung. The implication is seen in Penilaian Kinerja Guru (PKG) planning where the teacher explores her career needs, in the implementation of PKB where the teacher tries her career strategy, and in the evaluation where the teacher reflects on PKB. Teacher career management appears in the aspects of planning, implementation, and evaluation of PKB
Analisis Kompetensi Profesional Guru Mata Pelajaran Pengantar Administrasi Di Jurusan Administrasi Perkantoran SMK YPLP PGRI 1 Makassar
Authors: Renrohan Fadly Fadlun , Saleh Sirajuddin , Niswaty Risma 
Published: November 2020

Penelitian ini bertujuan untuk mengetahui kompetensi profesional guru Pelajaran Pengantar Administrasi Jurusan Administrasi Perkantoran SMK YPLP PGRI 1 Makassar. Penelitian ini menggunakan jenis penelitian kualitatif dengan pendekatan deskriptif kualitatif. Informan dalam penelitian ini sebanyak 5 (lima) orang. Pengumpulan data dilakukan melalui teknik wawancara, observasi, dan dokumentasi. Data yang telah dikumpulkan diolah dengan menggunakan teknik analisis yaitu reduksi data, penyajian data dan kesimpulan atau verifikasi. Hasil penelitian menunjukkan bahwa kompetensi profesional guru mata pelajaran pengantar administrasi jurusan Administrasi Perkantoran SMK YPLP PGRI 1 Makassar pada umunya sudah terlaksana dengan baik. Hal ini dinilai dari kelima indikator kompetensi profesional guru seperti, menguasai landasan pendidikan, yakni mampu memahami tujuan pendidikan untuk mencapai tujuan pendidikan nasional, mengusai bahan pengajaran, dengan menguasai bahan pengajaran kurikulum dan penyusunan program pengajaran, juga telah optimal dalam menetapkan tujuan pembelajaran, kemudian melaksanakan program pengajaran, dimana telah menciptakan iklim belajar mengajar yang tepat, dan menilai hasil dan proses belajar mengajar yang telah dilaksanakan dengan baik. Jadi kompetensi profesional guru mata pelajaran pengantar administrasi jurusan adminsitrasi perkantoran SMK YPLP PGRI 1 Makassar telah terimplementasi dengan baik.
MOTIVASI PEMANFAATAN E-LEARNING PADA GURU DI SEKOLAH KABUPATEN GOWA
Authors: Suhaeb Sutarsi 
Published: November 2020

The objectives to be achieved in this Partnership Program are: (1) Teachers and students use the internet as a public communication tool that optimally supports the learning process. (2) Using moodle learning as an interactive forum for students (3) Teachers have knowledge of how to build moodle-based e-learning classes. The expected outputs from community services are: (1) 20 Teachers understand the basic concepts of e-learning so that they can go further in applying online-based computer technology. (2) 20 teachers are motivated to use e-learning as a medium for the learning process. (3) 20 Teachers can apply e-learning so that students can be active in elaborating information obtained creatively and skillfully without being limited by time and space. The results in implementing community services consist of: (1) Participants can design e-learning classes using moodle. (2) Participants are able to manage classes in the learning process using E-learning. (3) Participants already have insight into E-learning. The conclusions of this PKM activity are: (1) Participants can design e-learning classes using moodle. (2) Participants have been able to manage classes in the learning process using E-learning. (3) the results of the study are based on the results of the distributed motivation questionnaire analysis, based on the results of the participant motivation questionnaire in SMP Negeri 1 Bontonompo 1, the success rate with a high category of 10 or 100%. Whereas Based on the results of the participant's motivation questionnaire in SMP Handayani, the success rate with a high category was 9 or 90%. While the success rate is in the medium category of 1 or 10%. Therefore the suggestion from this activity is that this activity should continue to be developed and applied widely among teachers because it can help increase motivation, effectiveness and efficient learning.
A Contemporary Analysis of Kant’s Concept of Goodwill and the Categorical Imperative
Authors: Ezedike Edward Uzoma 
Published: November 2020

This paper makes a contemporary appraisal of the concepts of the ‘Goodwill’ and the ‘Categorical Imperative’ in Kant’s formalistic, deontological ethics. Kant posits that the rightness of an act does not depend at all on the value of its consequences. For him, in order to know whether an act is right or wrong, we need only see whether it is in accordance with a valid moral rule. The test for a valid moral rule, as he conceives it, is purely formalistic. For a moral rule to be valid, it must pass the test of the foundational, supreme principle or ultimate criterion of morality, which Kant calls the “Categorical Imperative”. On this score, the paper, seeks to address the problem of ethical formalism and foundationalism associated with Kant’s theory in view of the contemporary challenge of ethical pluralism and destructive postmodernism. The objective of the paper is to reconcile with Kantianism with the contemporary shift from moral foundationalism and universalism to anti-foundationalism and relativism.
Sekolah Berbasis Boarding School dalam Pembentukan Karakter Sosial Peserta Didik
Authors: Salwia Salwia , Gani Hamsu Abdul , Suardi Suardi 
Published: November 2020

Penelitian ini bertujuan untuk mengetahui 1) Pelaksanaan kegiatan sekolah berbasis boarding school dalam pembentukan karakter sosial peserta didik di SMA Negeri 17 Makassar. 2) Karakter sosial peserta didik yang mengikuti boarding school di SMA Negeri 17 Makassar. Penelitian ini menggunakan penelitian kualitatif yang melibatkan 15 orang sebagai informan (10 orang peserta didik, 4 orang pembina asrama, dan kepala sekolah). Data penelitian ini dikumpulkan melalui observasi, wawancara, dan dokumentasi. Teknik pengabsahan data yaitu triangulasi sumber dan triangulasi teknik. Hasil penelitian menunjukkan bahwa (1) Pelaksanaan kegiatan sekolah berbasis boarding school dalam pembentukan karakter sosial peserta didik di SMA Negeri 17 Makassar meliputi bimbingan keagamaan, pengembangan bakat dan minat, keterampilan sikap prososial dan bimbingan belajar. Selain kegiatan asrama juga terdapat penerapan tata tertib, pemberian hukuman yang konsisten sesuai dengan tingkat pelanggaran sebagai rangkain pelaksanaan kegiatan sekolah. (2) Karakter sosial peserta didik yang mengikuti boarding school di SMA Negeri 17 Makassar dapat dilihat dari segi kejujuran kurang baik karena masih terdapat peserta didik yang tidak memiliki perilaku jujur. Dari segi toleransi, peserta didik saling menghargai dan menghormati tanpa membeda-bedakan keyakinan. Dari segi demokratis, pengambilan keputusan selalu diputuskan bersama. Dari segi semangat kebangsaan, selalu memperingati hari nasional. Dari segi peduli lingkungan, selalu menjaga kebersihan dan keamanan asrama. dari segi peduli sosial, melaksanakan kegiatan baksos. Dari segi tanggung jawab, melaksanakan dengan baik segala kegiatan dan dari segi kerjasama, peserta didik saling membantu melakukan segala kegiatan seperti kerja bakti.
Pelaksanaan Tugas Lurah Dalam Pelayanan Masyarakat Di Kelurahan Tompobalang Kecamatan Somba Opu Kabupaten Gowa
Authors: Adirudini Muh. , Rifdan Rifdan , Sailan Manan 
Published: November 2020

Penelitian ini bertujuan untuk mengkaji dan mendeskripsikan (1) Pelaksanaan tugas Lurah dalam pelayanan masyarakat di Kelurahan Tompobalang Kecamatan Somba Opu Kabupaten Gowa. (2) Faktor determinan dalam pelayanan masyarakat di Kelurahan Tompobalang Kecamatan Somba Opu Kabupaten Gowa. (3) Strategi pelaksanaan tugas Lurah dalam pelayanan masyarakat di Kelurahan Tompobalang Kecamatan Somba Opu Kabupaten Gowa. Jenis penelitian ini adalah penelitian kualitatif dengan pendekatan fenomenologi, dimana jenis penelitian ini dilakukan dengan mengamati subjek dan objek yang akan diteliti berdasarkan fakta-fakta yang ada. Fakta yang ditemukan dikaji dan dideskripsikan secara mendalam. Teknik pengumpulan data menggunakan observasi, wawancara, dokumentasi, dan dianalisis dengan teknik triangulasi. Hasil penelitian ini menunjukkan bahwa: (1) Pelaksanaan tugas lurah dalam pelayanan masyarakat di Kelurahan Tompobalang mengenai pola-pola tugas Lurah yang efektif dan efesien oleh pemerintah Kelurahan Tompobalang didapati bahwa pola-pola tugas Lurah Tompobalang sudah cukup efektif dan efesien, dimana Lurah selalu mengarahkan dalam memberikan tugas dan arahan yang dapat dimengerti walaupun Lurah tidak selalu berada di kantor dan bertugas diluar, namun dengan kecanggihan teknologi handphone hal tersebut dapat diatasi. (2) Faktor determinan yang dihadapi dalam pelaksanaan tugas lurah adalah sarana dan prasana yang belum memadai sehingga pelayanan masyarakat pun kurang optimal. (3) Strategi pelaksanaan tugas lurah dalam pelayanan masyarakat di Kelurahan Tompobalang yang dilakukan dengan Peningkatan kualitas penyelenggaraan pemerintahan dengan menerapkan Kebijaka Optimalisasi penerapan SOP (Standar Operasional Prosedur) dengan penguatan kualitas dukungan administrasi sarana,dan prasarana di kelurahan Tompobalang.
Meningkatkan Kemampuan Guru Mata Pelajaran Melalui Penerapan Model Pembelajaran Problem Solving: Upaya peningkatan kemampuan berfikir kritis Melalui Kegiatan Supervisi Akademik di SMA Negeri 1 Nunukan
Authors: Naim Khoirul 
Published: November 2020

Abstrak. Tujuan Penelitian ini untuk meningkatkan (1) kemampuan guru mata pelajaran dalm menyusun RPP Model pembelajaran problem solving melalui kegiatan suvervisi akademik; (2) kemampuan guru dalam menerapkan model pembelajaran problem solving sebagai upaya meningkatkan kemampuan berifikir kritis siswa melalui kegiatan supervisi akademik. Metode penelitian ini adalah penelitian tindakan sekolah yang dilaksanakan dua siklus dengan tahapan mulai dari perencanaan, pelaksanaan, pengamatan dan refleksi, penelitian melibatkan 5 guru SMA N 1 Nunukan. Hasil penelitian Dari hasil penelitian dapat disimpulkan bahwa (1) terjadi peningkatan kemampuan guru dalam menyusun RPP Model pembelajaran dari pra siklus dengan nilai 62,90 atau berada pada kategori kurang, kemudian di siklus I sebesar 77,27% dengan kategori cukup dan terjadi peningkatan hingga siklus II sebesar 95,11% dengan kategori Baik; (2) Penerapan metode pembelajaran problem solving melalui kegiatan akademik secara klinis dapat meningkatkan model pembelajaran guru karena dari pencapaian mulai dari pra siklus 31,46 % masih berada pada kategori kurang, di siklus I pencapaian sebesar 71,02% dengan kategori cukup dan terjadi peningkatan signifikan pada siklus II sebesar 91,50 atau dengan kategori baik. Keberhasil guru dalam mengimplementasikan metode pembelajaran akan membuat siswa memiliki sikap kritis dalam belajar mata pelajaran sains, yang berdampak pada peningkatan hasil belajarnya.
Bimbingan Pembuatan dan Penggunaan Alat Peraga Permainan Olahraga Tradisional Bagi Guru SDN 95 Bontobulaeng Kecamatan Bulukumpa Kabupaten Bulukumba
Authors: Lutfi Lutfi , Makkasau Andi , Nasaruddin Nasaruddin 
Published: November 2020

Permasalahan sekolah mitra adalah terbatasnya media pembelajaran yang kini digunakan, baik kuantitas maupun kualitasnya Pengabdian masyarakat bertujuan untuk (1) mempergunakan Alat Peraga Permainan Olahraga Tradisiona, Seni dan IPA dalam melaksanakan pembelajaran sesuai materi yang diajarkan untuk meningkatkan efesiensi waktu dalam proses pembelajaran, (2) meningkatkan kemapuan penguasaan materi dan keterampilan menggunakan alat peraga Permainan Olahraga Tradisiona, Seni dan IPA bagi Guru-guru di SDN 95 Bontobulaeng Kecamatan Bulukumpa Kabupaten Bulukumba. Selain itu dapat pula meningkatkan motivasi dalam proses belajar mengajar dan untuk menunjang pelaksanaan cara belajar siswa aktif (CBSA). Target khusus yang ingin dicapai adalah (1) menambah pengetahuan dan keterampilan serta kreatifitas guru menggunakan alat praktik alat peraga dalam percobaan, (2) mengembangkan media pembelajaran berdasarkan kebutuhan belajar dan perkembangan anak, serta (3) teratasinya kesulitan/keterbatasan penggunaan alat peraga Permainan Olahraga Tradisional, Seni dan IPA dalam percobaan untuk keperluan pembelajaran. Metode dan pendekatan yang digunakan yaitu: metode ceramah bervariasi demonstrasi, dan pemberian tugas dengan penerapan pendekatan Keterampilan Proses. Metode pengabdian ini menggunakan metode seminar dan pelatihan. Hasil pengabdian ini adalah guru mampu membuat Alat peraga Permainan Olahraga Tradisional, alat perga IPA, dan alat peraga Seni rupa SD. Implikasi pengabdian ini adalah sebagai acuan oleh praktisi pendidikan untuk meningkatkan keterampilan guru dalam mengembangkan permainan tradisional yang terintegrasi dalam pembelajaran di SD.