The mucopolysaccharidoses represent a devastating group of lysosomal storage diseases
affecting approximately 1 in 25 000 individuals. Advances in biochemistry and genetics over …

Research and drug developments fostered under orphan drug product development
programs have greatly assisted the introduction of efficient and safe enzyme-based …

The three iodothyronine selenodeiodinases catalyze the initiation and termination of thyroid
hormone effects in vertebrates. Structural analyses of these proteins have been hindered by …

Hemicelluloses and Lignin in Biorefineries provides an understanding of lignocellulosic
biomass, which is mainly composed of cellulose, hemicelluloses, and lignin. It promotes the …

In this study we have investigated a group of 29 Brazilian patients, who had been diagnosed
with the lysosomal storage disorder, Mucopolysaccharidosis type I (MPS-I). MPS I is caused …

α-l-Iduronidase is a glycosyl hydrolase involved in the sequential degradation of the
glycosaminoglycans heparan sulphate and dermatan sulphate. A deficiency in α-l …

Background Enzyme-replacement therapy has been assessed as a treatment for patients
who have mucopolysaccharidosis I (α-L-iduronidase deficiency). We aimed to investigate …

1, 4-β-d-Xylan is the major component of plant cell-wall hemicelluloses. β-d-Xylosidases are
involved in the breakdown of xylans into xylose and belong to families 3, 39, 43, 52, and 54 …

Restricting transgene expression to maturing erythroid cells can reduce the risk for activating
oncogenes in hematopoietic stem cells (HSCs) and their progeny, yet take advantage of …

Mucopolysaccharidosis I is a lysosomal storage disorder caused by a deficiency of the
lysosomal hydrolase α-l-iduronidase, which is required for the degradation of heparan …