Publications by authors named "Ah-Ra Ko"

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The efficacy of intracerebroventricular idursulfase-beta enzyme replacement therapy in mucopolysaccharidosis II murine model: heparan sulfate in cerebrospinal fluid as a clinical biomarker of neuropathology.
Young Bae Sohn, Ah-Ra Ko, Mi-Ran Seong, Soyeon Lee, Mi Ra Kim, Makoto Sakaguchi, Motomichi Kosuga

J Inherit Metab Dis· November 2018

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A Liquid Chromatography-Quadrupole-Time-of-Flight Mass Spectrometric Assay for the Quantification of Fabry Disease Biomarker Globotriaosylceramide (GB3) in Fabry Model Mouse.
Seok-Ho Shin, Min-Ho Park, Jin-Ju Byeon, Byeong Ill Lee, Yuri Park, Ah-Ra Ko

Pharmaceutics· June 2018

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BGN Mutations in X-Linked Spondyloepimetaphyseal Dysplasia.
Sung Yoon Cho, Jun-Seok Bae, Nayoung K D Kim, Francesca Forzano, Katta Mohan Girisha, Ah-Ra Ko, Noriko Miyake

Am J Hum Genet· June 2016

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AAV8-mediated expression of N-acetylglucosamine-1-phosphate transferase attenuates bone loss in a mouse model of mucolipidosis II.
Ah-Ra Ko, Dong-Kyu Jin, Sung Yoon Cho, Sung Won Park, Malgorzata Przybylska

Mol Genet Metab· April 2016

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Pharmacokinetics, Pharmacodynamics, and Efficacy of a Novel Long-Acting Human Growth Hormone: Fc Fusion Protein.
Su Jin Kim, Hyun-Hee Kwak, Sung Yoon Cho, Young Bae Sohn, Sung Won Park, Ah-Ra Ko

Mol Pharm· October 2015

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Effect of systemic high dose enzyme replacement therapy on the improvement of CNS defects in a mouse model of mucopolysaccharidosis type II.
Sung Yoon Cho, Jeehun Lee, Ah-Ra Ko, Min Jung Kwak, Sujin Kim

Orphanet J Rare Dis· October 2015

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Decreased performance in IDUA knockout mouse mimic limitations of joint function and locomotion in patients with Hurler syndrome.
Chihwa Kim, Min Jung Kwak, Sung Yoon Cho, Ah-Ra Ko, Jinguen Rheey

Orphanet J Rare Dis· September 2015

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Morphological, Molecular, and Biochemical Characterization of Monounsaturated Fatty Acids-Rich Chlamydomonas sp. KIOST-1 Isolated from Korea.
Seon-Mi Jeon, Ji Hyung Kim, Taeho Kim, Areumi Park, Ah-Ra Ko

J Microbiol Biotechnol· May 2015

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Morphological, molecular, and biochemical characterization of astaxanthin-producing green microalga Haematococcus sp. KORDI03 (Haematococcaceae, Chlorophyta) isolated from Korea.
Ji Hyung Kim, Abu Affan, Jiyi Jang, Mee-Hye Kang, Ah-Ra Ko

J Microbiol Biotechnol· February 2015

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Heterozygous mutations in cyclic AMP phosphodiesterase-4D (PDE4D) and protein kinase A (PKA) provide new insights into the molecular pathology of acrodysostosis.
Tadashi Kaname, Chang-Seok Ki, Norio Niikawa, George S Baillie, Jonathan P Day, Ah-Ra Ko, Kumiko Yanagi, Ko-Ichiro Yoshiura, Tatsuro Kondoh

Cell Signal· November 2014

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Improvement of cardiac function by short-term enzyme replacement therapy in a murine model of cardiomyopathy associated with Hunter syndrome evaluated by serial echocardiography with speckle tracking 2-D strain analysis.
Sang-Chol Lee, Jieun Lee, Dong-Kyu Jin, Jung-Sun Kim, Eun-Seok Jeon, Ah-ra Ko

Mol Genet Metab· July 2014

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A biochemical and physicochemical comparison of two recombinant enzymes used for enzyme replacement therapies of hunter syndrome.
Yo Kyung Chung, Young Bae Sohn, Jong Mun Sohn, Jieun Lee, Mi Sun Chang, Ah-Ra Ko

Glycoconj J· May 2014

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Calpain-mediated proteolysis of polycystin-1 C-terminus induces JAK2 and ERK signal alterations.
Hyunho Kim, Ah-Young Kang, Ah-ra Ko, Hayne Cho Park, Insuk So

Exp Cell Res· January 2014

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Therapeutic effects of repetitive transcranial magnetic stimulation in an animal model of Parkinson's disease.
Ji Yong Lee, Sung Hoon Kim, Ah-Ra Ko, Jin Suk Lee, Ji Hea Yu

Brain Res· November 2013

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High-dose enzyme replacement therapy attenuates cerebroventriculomegaly in a mouse model of mucopolysaccharidosis type II.
So Yoon Ahn, Yun Sil Chang, Dong Kyung Sung, Ah-Ra Ko, Chi Hwa Kim

J Hum Genet· November 2013

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Improvement of CNS defects via continuous intrathecal enzyme replacement by osmotic pump in mucopolysaccharidosis type II mice.
Young Bae Sohn, Jeehun Lee, Sung Yoon Cho, Su Jin Kim, Ah-Ra Ko

Am J Med Genet A· May 2013

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Five novel mutations of GALNS in Korean patients with mucopolysaccharidosis IVA.
Hyung-Doo Park, Ah-Ra Ko, Chang-Seok Ki, Soo-Youn Lee, Jong-Won Kim

Am J Med Genet A· March 2013

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Phase I/II clinical trial of enzyme replacement therapy with idursulfase beta in patients with mucopolysaccharidosis II (Hunter syndrome).
Young Bae Sohn, Sung Yoon Cho, Sung Won Park, Su Jin Kim, Ah-Ra Ko

Orphanet J Rare Dis· March 2013

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Clinical, biochemical, and genetic analysis of two korean patients with trichorhinophalangeal syndrome type I and growth hormone deficiency.
Young Bae Sohn, Chang-Seok Ki, Sung Won Park, Sung-Yoon Cho, Ah-Ra Ko

Ann Clin Lab Sci· February 2013

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LIN28B polymorphisms are associated with central precocious puberty and early puberty in girls.
Sung Won Park, Seung-Tae Lee, Young Bae Sohn, Sung Yoon Cho, Se-Hwa Kim, Ah-Ra Ko

Korean J Pediatr· October 2012

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Auditory characteristics and therapeutic effects of enzyme replacement in mouse model of the mucopolysaccharidosis (MPS) II.
Sung Hwa Hong, Hosuk Chu, Ki Ryung Kim, Moon Hee Ko, See Youn Kwon, Ah-Ra Ko

Am J Med Genet A· September 2012

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A Korean patient with Morquio B disease with a novel c.13_14insA mutation in the GLB1 gene.
Young Bae Sohn, Hyung-Doo Park, Sung Won Park, Se-Hwa Kim, Sung-Yoon Cho, Ah-Ra Ko

Ann Clin Lab Sci· June 2012

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Enzyme replacement therapy improves joint motion and outcome of the 12-min walk test in a mucopolysaccharidosis type VI patient previously treated with bone marrow transplantation.
Young Bae Sohn, Sung Won Park, Se-Hwa Kim, Sung-Yoon Cho, Sun-Tae Ji, Ah-Ra Ko

Am J Med Genet A· May 2012

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A study of the relationship between clinical phenotypes and plasma iduronate-2-sulfatase enzyme activities in Hunter syndrome patients.
Ok Jeong Lee, Su-Jin Kim, Young Bae Sohn, Hyung-Doo Park, Soo-Youn Lee, Ah-Ra Ko

Korean J Pediatr· March 2012

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A polymorphism in the growth hormone receptor is associated with height in children with Prader-Willi syndrome.
Sung Won Park, Seung-Tae Lee, Young Bae Sohn, Se Hwa Kim, Sung-Yoon Cho, Ah-Ra Ko

Am J Med Genet A· December 2011

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