144,303 research outputs found
Identification and Functional Analysis of Two New De Novo KCNMA1 Variants Associated with Liang-Wang syndrome.
No full textAIM
Loss-of-function KCNMA1 variants cause Liang-Wang syndrome (MIM #618729), a newly-identified multiple malformation syndrome with a broad spectrum of developmental and neurological phenotypes. However, the full spectrum of clinical features and underlying pathogenic mechanisms need full elucidation.
METHODS
Exome sequencing was used to identify pathogenic variants. Patch-clamp recordings were performed to access the effects of KCNMA1 variants on BK channels. Total and membrane protein expression levels of BK channels were characterized using Western blotting.
RESULTS
We report identification and functional characterization of two new de novo loss-of-function KCNMA1 variants p.(A172T) and p.(A314T) with characteristics of Liang-Wang syndrome. Variant p.(A172T) is associated with developmental delay, cognitive impairment, and ataxia. Mechanistically, p.(A172T) abolishes BK potassium current, inhibits Mg2+ -dependent gating, but shifts conductance-voltage (G-V) curves to more positive potentials when complexed with WT channels. Variant p.(A314T) is associated with developmental delay, intellectual disability, cognitive impairment, mild ataxia, and generalized epilepsy, suppresses BK current amplitude, and shifts G-V curves to more positive potentials when expressed with WT channels. In addition, two new patients with previously reported gain-of-function variants p.(N536H) and p.(N995S) are found to show epilepsy and paroxysmal dyskinesia as reported previously, but also exhibit additional symptoms of cognitive impairment, and dysmorphic features. Furthermore, variants p.(A314T) and p.(N536H) reduced total and membrane levels of BK proteins.
CONCLUSION
Our findings identified two new loss-of-function mutations of KCNMA1 associated with Liang-Wang syndrome, expanded the spectrum of clinical features associated with gain-of-function KCNMA1 variants, and emphasized the overlapping features shared by gain-of-function and loss-of-function mutations
Walchia (Walchia) fenga Zhao and Liang 1989
No full textWalchia (Walchia) fenga Zhao and Liang, 1989: PALPublished as part of Nielsen, David H., Robbins, Richard G. & Rueda, Leopoldo M., 2021, Annotated world checklist of the Trombiculidae and Leeuwenhoekiidae (1758 - 2021) (Acari: Trombiculoidea), with notes on nomenclature, taxonomy, and distribution, pp. 1-243 in Zootaxa 4967 (1) on page 37, DOI: 10.11646/zootaxa.4967.1.1, http://zenodo.org/record/474551
Hubungan populasi kelelawar (Chiroptera) dengan komunitas Arthropoda tanah di Gua Liang Boeh, Liang Seungit dan Sarongge Kabupaten Tasikmalaya
No full textINDONESIA :
Kelelawar adalah komponen penting penghasil guano yang merupakan salah satu sumber makanan utama di ekosistem gua, termasuk bagi kelompok Arthropoda tanah. Tujuan penelitian ini adalah untuk mengetahui hubungan populasi kelelawar dengan komunitas Arthropoda tanah di Gua Liang Boeh, Liang Seungit dan Sarongge di Kabupaten Tasikmalaya. Penelitian dilaksanakan pada bulan Januari-Maret 2019. Metode pengumpulan data populasi kelelawar dilakukan pada pukul 17.00-18.00 WIB, dengan pemasangan jaring mis-net dan dihitung menggunakan Hand Counter. Sampel guano ditampung selama 24 jam dengan 3x pengulangan menggunakan plastik berukuran 5x4 m dan diuji kandungan senyawa kimia parameter N dan P. Koleksi Arthropoda tanah menggunakan Hand Collecting, Pitfall Trap, dan ekstraksi tanah corong Berlesse. Faktor abiotik yang diukur yaitu intensitas cahaya, suhu udara, suhu tanah, kelembapan udara, kelembapan tanah dan pH tanah. Data dianalisis menggunakan uji korelasi Rank Spearmen. Hasil penelitian menunjukkan populasi kelelawar paling banyak adalah Gua Sarongge yaitu 1193 individu, Liang Boeh 188, dan paling rendah yaitu Liang Seungit 471 individu. Berat Guano yang dihasilkan Sarongge sebanyak 222 g, Liang Boeh 148 g, Liang Seungit paling rendah yaitu 10 g. Arthropoda yang paling banyak ditemukan adalah di Gua Sarongge terdapat 22 jumlah spesies, jumlah kelimpahan 4652 individu, Liang Boeh 13 spesies, jumlah kelimpahan 11177 individu, sedangkan yang paling rendah adalah Gua Liang Seungit jumlah 12 spesies dan memiliki kelimpahan 157 individu. Korelasi antara populasi kelelawar, berat guano, jumlah spesies dan kelimpahan individu Arthropoda mempunyai korelasi positif dan sangat kuat.
ENGLISH :
Bats are an important component of producing guano which is one of the main food sources in eco-cave including the group of soil arthropods. The purpose of this study was to determine the relationship between the population of Bats and the Arthropod community in Liang Boeh Cave, Liang Seungit and Sarongge in Tasikmalaya District. The study was conducted in January-March 2019. The method of collecting data on bat populations was carried out in dark zones at 17.00-18.00 WIB, with the installation of mis-net nets and calculated using Hand Counter. Guano samples were collected for 24 hours with 3x repetitions using 5x4 m plastic and tested the chemical compounds N and P parameters. Soil Arthropod collection used Hand Collecting, Pitfall Trap, and Funnel Extract (Berlesse). Abiotic factors measured are light intensity, air temperature, soil temperature, air humidity, soil moisture and soil pH. Data were analyzed using version 23 spss statistics of rank spearmen correlation test. The results showed that the largest population of bats was Cave Sarongge as many as 1193 individuals, Liang Boeh 188, and the lowest number, namely Liang as much as 471 individuals. The weight of Guano produced by Sarongge was 222 g, Liang boeh 148 g, the lowest hole was 10 g. The most abundant arthropods found in the Sarongge Cave are 22 species, the total abundance of 4652 individuals, the total number of 13 species, the abundance of 11177 individuals, while the lowest is the Liang Seungit Cave with 12 species and an abundance of 157 individuals. Correlation between bat population, guano weight, number of species and abundance of individual arthropods have a positive and very strong correlation
Gelastyrella yunnanensis Ran et Liang, sp. nov.
No full text3. Gelastyrella yunnanensis Ran et Liang, sp. nov. (Figures 9–16) Length: male 5.2 mm, female 5.6 mm. Male: General color dull brown. Vertex (Fig. 9) dark brown, with a broad yellow brown longitudinal stripe in middle, anterior margin acutely roundly produced, posterior margin anteriorly angularly produced, lateral margins almost parallel. Frons (Fig. 10) dark brown, median carina distinct, the area between submedian carinae and the anterior margin of vertex black. Clypeus black brown, with yellow brown spots in base. Antenna dull brown. Pronotum black brown; anterior margin roundly produced slightly between eyes; with a small pit beyond middle line, and with yellow brown spots and protuberance on disc. Mesonotum triangular, with black brown spots on disc. Tegmina (Fig. 11) with veinlets among longitudinal veins, veins slightly green. Legs yellow brown, with black brown spots. Spinal formula of hind legs: 718 2; 721 2. Male genitalia: Anal segment (Fig. 13) in dorsal view nearly oval, anal foramen lying in middle. Genital styles (Fig. 14) triangular, base convex hooklike. Aedeagus (Figs. 15, 16) with two anterodorsally directed processes ventrally near base; apex with two posterodorsally directed processes dorsally. Female: General color paler than that of male. Spinal formula of hind legs: 721 2. Materials examined Holotype: male, China: Yunnan, Xishuangbanna, Xiaomengyang, 850 m, 5. ix. 1958 (L. Y. Zheng) (IZCAS). Paratype. 1 female, China: Yunnan, Xishuangbanna, Mengla, 620650 m, 29. v. 1959 (F. J. Pu) (IZCAS). Distribution China: Yunnan. Remarks This species is similar to G. litaoensis Yang, but can be separated from the latter by vertex dark brown, with a broad yellow brown longitudinal line in middle (vertex grayish with basal portion somewhat green in G. litaoensis Yang); spinal formula of hind legs: male 7 (1821) 2; female 721 2 (spinal formula of hind legs: 735 2 in G. litaoensis Yang). Etymology The species is named for where it was collected.Published as part of Ran, Hong-Fan & Liang, Ai-Ping, 2006, The issid genus Gelastyrella Yang (Hemiptera: Fulgoroidea: Issidae) from China, pp. 63-68 in Zootaxa 1238 on page 67, DOI: 10.5281/zenodo.17282
KARAKTERISTIK EDIBLE FILM SODIUM CASEINATE LIANG-TEH KAYA ANTIOKSIDAN
No full textIn general, food packaging materials are divided into two major groups: bio and non-biodegradable. Currently being developed is biodegradable packaging, including edible films. The application of edible films as packaging with carriers of antioxidants and colorants. One alternative of natural antioxidant to applied in edible films is liang-tea. The liang-tea contains of alkaloids, flavonoids, phenols, and tannins, that contribute antioxidant activity. This research aims to determine the concentration of infusion liang-tea that produces the best characteristics of sodium caseinate edible film that rich antioxidant. The research used a randomized block design with a single treatment of the concentration of infused liang-tea of 0, 25, 50, 75, 100, 125, and 150% (v/w, of sodium caseinate), with 4 replications. The result indicates that the concentration of 150% (v/w) of sodium caseinate of infused liang-tea produced the best rich antioxidant edible films. The edible film had a thickness 0.20 mm, solubility 13.66%, L* colour value 88.39, a* colour value of -0.23, b* colour value of 5.01, water holding capacity 3.47 (g/g), moisture content 14.11%, and antioxidant activity 41.42%.Secara umum bahan kemasan pangan dibagi dalam dua kelompok besar yaitu bahan kemasan non-biodegradable dan biodegradable. Saat ini terus dilakukan pengembangan yaitu kemasan biodegradable, di antaranya dalam bentuk edible film. Edible film diterapkan sebagai kemasan dengan pembawa antioksidan dan pewarna. Salah satu alternatif antioksidan alami yang dapat diaplikasikan pada edible film adalah liang-teh yang merupakan bahan alam kaya antioksidan. Liang-teh mengandung alkaloid, flavonoid, fenol, dan tanin, yang berkontribusi aktivitas antioksidan. Penelitian ini bertujuan untuk menentukan konsentrasi infusa liang-teh yang menghasilkan karakteristik edible film sodium caseinate terbaik yang kaya antioksidan. Penelitian ini menggunakan Rancangan Acak Kelompok dengan satu faktor perlakuan, yaitu konsentrasi infusa liang-teh yang terdiri dari 0, 25, 50, 75, 100, 125, dan 150% (v/b, sodium caseinate), dengan 4 ulangan. Hasil penelitian menunjukkan bahwa konsentrasi 150% (v/b) sodium caseinate dari infusa liang-teh menghasilkan edible film kaya antioksidan terbaik. Edible film memiliki ketebalan sebesar 0,20 mm, solubilitas 13,66%, nilai warna L* 88,39, nilai warna a* -0,23, nilai warna b* 5,01, Water Holding Capacity 3,47 (g/g), kadar air 14,11% dan aktivitas antioksidan 41,42%
KARAKTERISTIK EDIBLE FILM SODIUM CASEINATE LIANG-TEH KAYA ANTIOKSIDAN
No full textIn general, food packaging materials are divided into two major groups: bio and non-biodegradable. Currently being developed is biodegradable packaging, including edible films. The application of edible films as packaging with carriers of antioxidants and colorants. One alternative of natural antioxidant to applied in edible films is liang-tea. The liang-tea contains of alkaloids, flavonoids, phenols, and tannins, that contribute antioxidant activity. This research aims to determine the concentration of infusion liang-tea that produces the best characteristics of sodium caseinate edible film that rich antioxidant. The research used a randomized block design with a single treatment of the concentration of infused liang-tea of 0, 25, 50, 75, 100, 125, and 150% (v/w, of sodium caseinate), with 4 replications. The result indicates that the concentration of 150% (v/w) of sodium caseinate of infused liang-tea produced the best rich antioxidant edible films. The edible film had a thickness 0.20 mm, solubility 13.66%, L* colour value 88.39, a* colour value of -0.23, b* colour value of 5.01, water holding capacity 3.47 (g/g), moisture content 14.11%, and antioxidant activity 41.42%.Secara umum bahan kemasan pangan dibagi dalam dua kelompok besar yaitu bahan kemasan non-biodegradable dan biodegradable. Saat ini terus dilakukan pengembangan yaitu kemasan biodegradable, di antaranya dalam bentuk edible film. Edible film diterapkan sebagai kemasan dengan pembawa antioksidan dan pewarna. Salah satu alternatif antioksidan alami yang dapat diaplikasikan pada edible film adalah liang-teh yang merupakan bahan alam kaya antioksidan. Liang-teh mengandung alkaloid, flavonoid, fenol, dan tanin, yang berkontribusi aktivitas antioksidan. Penelitian ini bertujuan untuk menentukan konsentrasi infusa liang-teh yang menghasilkan karakteristik edible film sodium caseinate terbaik yang kaya antioksidan. Penelitian ini menggunakan Rancangan Acak Kelompok dengan satu faktor perlakuan, yaitu konsentrasi infusa liang-teh yang terdiri dari 0, 25, 50, 75, 100, 125, dan 150% (v/b, sodium caseinate), dengan 4 ulangan. Hasil penelitian menunjukkan bahwa konsentrasi 150% (v/b) sodium caseinate dari infusa liang-teh menghasilkan edible film kaya antioksidan terbaik. Edible film memiliki ketebalan sebesar 0,20 mm, solubilitas 13,66%, nilai warna L* 88,39, nilai warna a* -0,23, nilai warna b* 5,01, Water Holding Capacity 3,47 (g/g), kadar air 14,11% dan aktivitas antioksidan 41,42%
Gelastyrella hainanensis Ran et Liang, sp. nov.
No full text2. Gelastyrella hainanensis Ran et Liang, sp. nov. (Figures 1– 8) Length: male 6.3 mm, female 7.5 mm. Male: General color dull brown. Vertex (Fig. 1) yellow brown, median carina feeble; anterior margin slightly acutely roundly produced, posterior margin anteriorly angularly produced, lateral margins almost parallel. Frons (Fig. 2) dark brown, median carina present; the area between submedian carinae and anterior margin of vertex black brown. Clypeus dark brown, paler in middle, laterally transversely obliquely striate. Antennae dark brown. Pronotum dull green brown; anterior margin slightly acutely produced between eyes; median carina obscure; with a fuscous pit beyond median carina, and with dark spots on disc. Mesonotum triangular, dark brown, with green shining; with median carina and lateral carinae. Tegmina (Fig. 3) with transverse veinlets among longitudinal veins, veins slightly green. Legs yellow brown, with dark brown stripes. Spinal formula of hind legs: 849 2. Male genitalia: Anal segment (Fig. 5) in dorsal view almost rounded in apical 1 / 3, long striped in basal 2 / 3, anal foramen lies in apical 1 / 3. Genital styles (Fig. 6) almost triangular, base convex like a hook. Aedeagus (Fig. 78) ventrally convex triangularly, near apex with two anteroventally directed processes, apex also with two processes dorsally. Female: General color is darker than that of male. Spinal formula of hind legs: 8 (40 42) 2. Materials examined Holotype. Male, China: Hainan, Qiongzhong, 20. iv. 1984 (S. L. Liu) (TMNH). Paratype. 1 female, China: Hainan, 14. v. 1934 (C. He) (IZCAS). Distribution China: Hainan. Remarks This species is similar to G. litaoensis Yang, but can be separated from the latter by vertex yellow brown (vertex grayish with basal portion somewhat green in G. litaoensis Yang); spinal formula of hind legs: male 849 2; female 8 (4042) 2 (spinal formula of hind legs: 735 2 in G. litaoensis Yang). Etymology This species is named for where it was collected.Published as part of Ran, Hong-Fan & Liang, Ai-Ping, 2006, The issid genus Gelastyrella Yang (Hemiptera: Fulgoroidea: Issidae) from China, pp. 63-68 in Zootaxa 1238 on pages 65-67, DOI: 10.5281/zenodo.17282
Purchasing power parity in G-7 countries: Further evidence based on ADL test for threshold cointegration
No full textThis study applies a newly-developed Autoregressive Distributed Lag (ADL) test for threshold cointegration, proposed by Li and Lee (2010) to test the validity of long-run purchasing power parity (PPP) for G-7 countries over the January 1994 to April 2010. The empirical results indicate that PPP only holds true for Canada and France two countries. Our results have important policy implications for the G-7 countries under study.Purchasing Power Parity; G-7 Countries; ADL Test; Threshold Cointegration
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