| Title |
A biochemical and physicochemical comparison of two recombinant enzymes used for enzyme replacement therapies of hunter syndrome
|
|---|---|
| Published in |
Glycoconjugate Journal, April 2014
|
| DOI | 10.1007/s10719-014-9523-0 |
| Pubmed ID | |
| Authors |
Yo Kyung Chung, Young Bae Sohn, Jong Mun Sohn, Jieun Lee, Mi Sun Chang, Younghee Kwun, Chi Hwa Kim, Jin Young Lee, Yeon Joo Yook, Ah-Ra Ko, Dong-Kyu Jin |
| Abstract |
Mucopolysaccharidosis II (MPS II, Hunter syndrome; OMIM 309900) is an X-linked lysosomal storage disease caused by a deficiency in the enzyme iduronate-2-sulfatase (IDS), leading to accumulation of glycosaminoglycans (GAGs). For enzyme replacement therapy (ERT) of Hunter syndrome, two recombinant enzymes, idursulfase (Elaprase®, Shire Human Genetic Therapies, Lexington, MA) and idursulfase beta (Hunterase®, Green Cross Corporation, Yongin, Korea), are currently available in Korea. To compare the biochemical and physicochemical differences between idursulfase and idursulfase beta, we examined the formylglycine (FGly) content, specific enzyme activity, mannose-6-phosphate (M6P) content, sialic acid content, and in vitro cell uptake activity of normal human fibroblasts of these two enzymes.The FGly content, which determines the enzyme activity, of idursulfase beta was significantly higher than that of idursulfase (79.4 ± 0.9 vs. 68.1 ± 2.2 %, P < 0.001). In accordance with the FGly content, the specific enzyme activity of idursulfase beta was significantly higher than that of idursulfase (42.6 ± 1.1 vs. 27.8 ± 0.9 nmol/min/μg protein, P < 0.001). The levels of M6P and sialic acid were not significantly different (2.4 ± 0.1 vs 2.4 ± 0.3 mol/mol protein for M6P and 12.3 ± 0.7 vs. 12.4 ± 0.4 mol/mol protein for sialic acid). However, the cellular uptake activity of the normal human fibroblasts in vitro showed a significant difference (Kuptake, 5.09 ± 0.96 vs. 6.50 ± 1.28 nM protein, P = 0.017).In conclusion, idursulfase beta exhibited significantly higher specific enzyme activity than idursulfase, resulting from higher FGly content. These biochemical differences may be partly attributed to clinical efficacy. However, long-term clinical evaluations of Hunter syndrome patients treated with these two enzymes will be needed to demonstrate the clinical implications of significant difference of the enzyme activity and the FGly content. |
X Demographics
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Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Unknown | 1 | 100% |
Demographic breakdown
| Type | Count | As % |
|---|---|---|
| Members of the public | 1 | 100% |
Mendeley readers
The data shown below were compiled from readership statistics for 53 Mendeley readers of this research output. Click here to see the associated Mendeley record.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Belgium | 1 | 2% |
| Unknown | 52 | 98% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Researcher | 21 | 40% |
| Student > Master | 9 | 17% |
| Other | 6 | 11% |
| Student > Ph. D. Student | 5 | 9% |
| Professor > Associate Professor | 3 | 6% |
| Other | 5 | 9% |
| Unknown | 4 | 8% |
| Readers by discipline | Count | As % |
|---|---|---|
| Biochemistry, Genetics and Molecular Biology | 19 | 36% |
| Agricultural and Biological Sciences | 14 | 26% |
| Medicine and Dentistry | 7 | 13% |
| Pharmacology, Toxicology and Pharmaceutical Science | 3 | 6% |
| Chemistry | 2 | 4% |
| Other | 3 | 6% |
| Unknown | 5 | 9% |
Attention Score in Context
This research output has an Altmetric Attention Score of 7. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 09 January 2024.
All research outputs
#5,240,751
of 25,374,917 outputs
Outputs from Glycoconjugate Journal
#98
of 929 outputs
Outputs of similar age
#48,389
of 241,759 outputs
Outputs of similar age from Glycoconjugate Journal
#2
of 8 outputs
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So far Altmetric has tracked 929 research outputs from this source. They receive a mean Attention Score of 3.8. This one has done well, scoring higher than 89% of its peers.
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