Disease relevance of Ids

• Mucopolysaccharidosis type II (MPS II, Hunter syndrome) is a congenital storage disorder resulting from mutations on the iduronate-2-sulfatase (IDS) gene [1].

High impact information on Ids

• The E1A mutants containing a deletion in either the N terminus or the conserved region 1 were unable to induce apoptosis in cooperation with Ids [2].
• Apoptosis also is induced in mouse A40 (p53-/-) cells by E1A alone or E1A plus Ids after transient transfection of the expression vectors [2].
• After induction of E1A and Ids, EId10 and EId23 cells began to accumulate in S phase and undergo apoptosis before entering G2 phase, suggesting that abnormal synthesis of DNA induced by coexpression of E1A, Id-1H, and Id-2H results in the induction of apoptosis [2].
• Thus, Ids are localized to different cell types and are differentially expressed, at both the mRNA and protein levels, along the epididymis [3].
• Uptake of recombinant iduronate-2-sulfatase into neuronal and glial cells in vitro [1].

Associations of Ids with chemical compounds

• Two different approaches to obtain recombinant IDS have been utilized: production of the recombinant enzyme by a transfected human clone (Bosc 23 cells); production of the recombinant enzyme by adenoviral transduction of neuronal (SK-N-BE) or glial (C6) cells [1].
• Our data indicate that the transfected as well as the infected cells produce a large amount of the IDS enzyme, which is efficiently endocytosed into neuronal and glial cells through the mannose 6-phosphate (M6P) receptor system [1].

Analytical, diagnostic and therapeutic context of Ids

• The E1A domains required for induction of apoptosis, analysed by transfection with expression vectors for E1A, Ids and their mutants, followed by flow cytometry, reside in N-terminal (positions 17 - 38), CR1 and CR2 regions [4].
• Somatic gene therapy appears therefore to be suitable to correct IDS deficiency in brain cells [1].

References