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在“Elsevier电子期刊”中，命中：159条，耗时：小于0.01 秒

在所有数据库中总计命中：343条

1.Comparative analysis of maple syrups and natural sweeteners: Carbohydrates composition and classification (differentiation) by HPAEC-PAD and FTIR spectroscopy-chemometrics

作者：Erika Mellado-Mojica^a ; Navindra P. Seeram^b ; Mercedes G. Ló ; pez^a ; ^{mlopez@ira.cinvestav.mx" class="auth_mail" title="E-mail the corresponding author}

关键词：AS ; agave syrup ; CS ; corn syrup ; DP ; degree of polymerization ; FOS ; fructooligosaccharides ; HB ; honey ; MOS ; maltooligosaccharides ; MS ; maple syrup ; SS ; sugarcane syrup ; MSS ; Acer saccharum sap

刊名：Journal of Food Composition and Analysis

出版年：2016

2.Plasma amino acid and urine organic acid profiles of Filipino patients with maple syrup urine disease (MSUD) and correlation with their neurologic features

作者：Mary Anne D. Chiong ; MD ; MSc^a ; ^{mdchiong1@up.edu.ph} ; Marilyn A. Tan^b ; Cynthia P. Cordero^c ; Esphie Grace D. Fodra^a ; Judy S. Manliguis^a ; Cristine P. Lopez^a ; Leslie Michelle M. Dalmacio^d

关键词：MSUD ; maple syrup urine disease ; BCAA ; branched chain amino acids ; LNAA ; large neutral amino acids ; BCKAD ; branched chain keto acid dehydrogenase

刊名：Molecular Genetics and Metabolism Reports

出版年：2016

3.Maple Syrup Urine Disease Complicated with Kyphoscoliosis and Myelopathy

作者：Jia-Woei Hou^a ; ^b ; ^{hou76@cgh.org.tw" class="auth_mail" title="E-mail the corresponding author}

关键词：acrodermatitis enteropathica ; DBT gene ; maple syrup urine disease ; myelopathy ; zinc deficiency

刊名：Pediatrics & Neonatology

出版年：2016

4.Identification of a novel BCKDHA deletion causing maple syrup urine disease

作者：Sara Bremer^a ; ^{sbremer@ous-hf.no" class="auth_mail" title="E-mail the corresponding author} ; Yngve Thomas Bliksrud^a ; Helge Rootwelt^a ; Berit Woldseth^a ; Trine Tangeraas^b ; Ingjerd Sæ ; ves^b ; Sara Sofie Viksmoen Watle^b

关键词：Maple syrup urine disease ; BCKDHA ; branched-chain amino acids ; newborn screening

刊名：Meta Gene

出版年：2016

5.Molecular analysis of maple syrup urine disease in Jordanian families

关键词：Maple syrup urine disease ; Branched-chain α-ketoacid dehydrogenase complex ; BCKDHA ; DBT ; Jordan

刊名：Meta Gene

出版年：2016

作者：Flavia Feier^a ; ^b ; Ida Vanessa D. Schwartz^c ; ^d ; Abigail R. Benkert^e ; Joao Seda Neto^f ; Irene Miura^f ; Paulo Chapchap^f ; Eduardo Antunes da Fonseca^f ; Sandra Vieira^g ; ^h ; Maria Lú ; cia Zanotelli^h ; Filippo Pinto e Vairo^c ; Jose Simon Camelo Jrⁱ ; Ana Vitoria Barban Marguttiⁱ ; George V. Mazariegos^j ; Erik G. Puffenberger^e ; ^k ; Kevin A. Strauss^e ; ^k ; ^l ; ^{kstrauss@clinicforspecialchildren.org" class="auth_mail" title="E-mail the corresponding author}

关键词：Maple syrup urine disease ; Branched chain ketoacid dehydrogenase ; Living related donor liver transplantation ; Domino transplantation

刊名：Molecular Genetics and Metabolism

出版年：2016

7.Bacillus amyloliquefaciens levansucrase-catalyzed the synthesis of fructooligosaccharides, oligolevan and levan in maple syrup-based reaction systems

作者：Mengxi Li ; Sooyoun Seo ; Salwa Karboune ; ^{salwa.karboune@mcgill.ca" class="auth_mail" title="E-mail the corresponding author}

关键词：Maple syrup ; Fructooligosaccharides ; Oligolevan ; Levan ; Levansucrase ; Acceptor specificity

刊名：Carbohydrate Polymers

出版年：2015

8.Challenges in diagnosis and counseling of a family with two recessive neurometabolic disorders

作者：Heba S. Abd-Elkhalek ; Radwa Gamal ; Osama K. Zaki ; Solaf M. Elsayed ; ^{elsayed683@yahoo.com" class="auth_mail" title="E-mail the corresponding author}

关键词：Consanguinity ; Inborn errors ; Neurometabolic ; Maple syrup ; Tay&ndash ; Sachs disease

刊名：Egyptian Journal of Medical Human Genetics

出版年：2016

9.Eleven novel mutations of the BCKDHA, BCKDHB and DBT genes associated with maple syrup urine disease in the Chinese population: Report on eight cases

作者：Xiyuan Li^a ; Yuan Ding^a ; Yupeng Liu^a ; Yanyan Ma^a ; Jinqing Song^a ; Qiao Wang^a ; Mengqiu Li^b ; Yaping Qin^b ; Yanling Yang^a ; ^{yanlingy@vip.sina.com" class="auth_mail" title="E-mail the corresponding author} ; ^{organic.acid@126.com" class="auth_mail" title="E-mail the corresponding author}

关键词：Maple syrup urine disease ; Branched chain amino acids ; BCKDHA gene ; BCKDHB gene ; DBT gene ; Prenatal diagnosis

刊名：European Journal of Medical Genetics

出版年：2015

10.Identification of six novel mutations in Iranian patients with maple syrup urine disease and their in silico analysis‿

作者：Maryam Abiri^a ; ^b ; Razieh Karamzadeh^c ; ^d ; Morteza Karimipoor^b ; Shirin Ghadami^b ; Mohammad Reza Alaei^e ; Samira Dabagh Bagheri^f ; Hamideh Bagherian^f ; Aria Setoodeh^g ; Mohammad Reza Noori-Daloii^a ; Sirous Zeinali^b ; ^f ; ^{zeinali@kawsar.ir" class="auth_mail" title="E-mail the corresponding author}

关键词：MSUD ; BCKDHA gene ; Iran ; Mutation analysis

刊名：Mutation Research/Fundamental and Molecular Mechanisms of Mutagenesis

出版年：2016

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