Several carriers were evaluated for use in the delivery of exogenous glucocerebrosidase to monocytes from Gaucher's disease patients. Only gamma globulin-coated, resealed erythrocytes proved to be an effective vehicle for enzyme delivery. Glucocerebrosidase added in this manner normalized intracellular enzyme levels for at least 18 hr. In this model system for the study of enzyme replacement th...

tyrosinase (ec: 1.14.18.1) is a copper - containing enzyme which is distributed in all domains of life such as prokaryote, eukaryote, mammals, invertebrates and plants. tyrosinase catalyzes the oxidation of monophenols to diphenols and diphenols to o-quinones . the tyrosinase crystallographic data shows two histidine -rich regions named cua and cub. a loop containing residues m374, s375 and v37...

Isolated chloroplast coupling factor 1 ATPase is known to retain about 1 mol of tightly bound ADP/mol of enzyme. Some experimental results have given evidence that the bound ADP is at catalytic sites, but this view has not been supported by observations of a slow replacement of the bound ADP when CaATP or MgATP is added. The experiments reported in this paper show why a slow replacement of ADP ...

objective: osteoporosis or silent disease is a major bone disorder in elderly women in current century. estrogen has an important role in osteogenesis and prevention of bone fractures. hormone replacement therapy (hrt) is usually accompanied by such effects as breast and ovary cancers. thus, there is an increasing demand for replacement with plant phytoestrogens. this study is focused on determ...

Mesenteric lymphadenopathy is a rare manifestation of Gaucher disease (GD) with only 26 cases reported worldwide and its outcome remains largely unknown. In this manuscript, we described a 17-year-old girl with GD who has been treated with standard enzyme replacement therapy (ERT) for 16 years. The follow-up of her mesenteric lymphadenopathy began 13 years ago, which is one of the longest follo...

We report on the successful desensitization of an adult female with Pompe disease who had previously experienced anaphylaxis to intravenous alglucosidase alfa therapy. The starting alglucosidase alfa dose for desensitization was 10mg/kg with gradual dose escalation and desensitization via serial dilution was completed over five infusions. This methodology serves as a means to desensitize patien...

The drugs target the pathological proteins for pharmacological intervention through their vunerability of chemophoric and congate groups 1-3 at therapeutic level. They are essential binding affinity, intrinsic efficacy bioavailability. introduced in bioactive molecules by replacement hydrogen atoms. implicated SAR modifications interactional participation with receptor or enzyme protein. influe...