Decreasing activity and altered protein processing of human iduronate-2-sulfatase mutations demonstrated by expression in COS7 cells

Biochem Genet. 2012 Dec;50(11-12):990-7. doi: 10.1007/s10528-012-9538-9. Epub 2012 Sep 19.

Authors

Ratana Charoenwattanasatien¹, James R Ketudat Cairns, Siriporn Keeratichamroen, Phannee Sawangareetrakul, Pranoot Tanpaiboon, Duangrurdee Wattanasirichaigoon, Suthipong Pangkanon, Jisnuson Svasti, Voraratt Champattanachai

Affiliation

¹ Laboratory of Biochemistry, Chulabhorn Research Institute, Bangkok, 10210, Thailand.

PMID: 22990955
DOI: 10.1007/s10528-012-9538-9

No abstract available

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Animals
COS Cells
Catalytic Domain
Chlorocebus aethiops
Enzyme Activation
Gene Expression Regulation, Enzymologic*
Glycoproteins / genetics*
Glycoproteins / metabolism
Glycosylation
Humans
Leukocytes / enzymology
Mucopolysaccharidosis II / genetics*
Mucopolysaccharidosis II / pathology
Mutation*
Plasmids / genetics
Plasmids / metabolism
Protein Conformation
Protein Folding
Severity of Illness Index
Transfection

Substances

Glycoproteins
IDS protein, human