The present invention pertains to a novel treatment of pathologies caused by an increased synthesis or accumulation of sulfolipids such as sulfatide. The invention provides mutated arylsulfatase A (ARSA or ASA, EC 3.1.6.8) enzymes with increased activity towards sulfatide metabolization. The invention provides nucleic acids encoding the mutant ARSA, the use of the proteins and nucleic acids, as well as pharmaceutical compositions comprising them, in the treatment of lysosomal storage disorders (LSDs) such as metachromatic leukodystrophy (MLD).

The present inventions relates to a method for the production of gellan gum, under mixing conditions, the method comprising a) providing a fermentation broth or other liquid medium containing gellan gum, b) if necessary adjusting the temperature and the pH of the fermentation broth/liquid medium to allow or facilitate enzymatic treatment in step c, c) adding one or more enzymes capable of reducing or abolishing the enzymatic activity of S. elodea derived arylsulfatase and/or -glucuronidase, said one or more enzymes being added in an amount sufficient to reduce or abolish the enzymatic activity of S. elodea derived arylsulfatase and/or -glucuronidase in the broth/liquid medium, and/or treating the broth/liquid medium at a temperature between 90 C. and 125 C. for a period of time sufficient to reduce or abolish the enzymatic activity of S. elodea derived arylsulfatase and/or -glucuronidase in the broth/liquid medium, and d) optionally recovering the gellan gum from the gellan gum containing broth/liquid medium.

Provided is a method for determining activity of arylsulfatase in an aqueous system, which comprises a step in which arylsulfatase is subjected to reaction with a substrate, from which fluorophore or chromophore is liberated by suffering an action of the arylsulfatase, in an aqueous reaction system having high ionic strength. Also, provided are a lactase preparation having a lactase activity of 4,000 NLU/g or more according to the FCC IV method and having an arylsulfatase activity of 0.1% or less of the lactase activity as the basis, in which the arylsulfatase activity has been determined by the method for determining activity of arylsulfatase in an aqueous system according to the fluorescence method of the present invention; a method for producing this preparation; and a dairy product which comprises using this preparation.

The present invention comprises a method of concentrating a composition comprising a polypeptide of interest and the use of such concentrated composition for the treatment of diseases in mammals, in particular by subcutaneous injection.

The present invention describes a intracellular produced lactase, which comprises less than 40 units arylsulfatase activity per NLU of lactase activity. The invention also provides a process comprising treating a substrate with an enzyme preparation, wherein the enzyme preparation is substantially free from arylsulfatase.

The present invention provides, among other things, improved methods for purifying arylsulfatase A (ASA) protein produced recombinantly for enzyme replacement therapy. The present invention is, in part, based on the surprising discovery that recombinant ASA protein can be purified from unprocessed biological materials, such as, ASA-containing cell culture medium, using a process involving as few as four chromatography columns and only one step of post-chromatographic ultrafiltration/diafiltration.

The present invention comprises a method of concentrating a composition comprising a polypeptide of interest and the use of such concentrated composition for the treatment of diseases in mammals, in particular by subcutaneous injection.

The present invention comprises a method of concentrating a composition comprising a polypeptide of interest and the use of such a concentrated composition for the treatment of diseases in mammals, in particular by subcutaneous injection.

Provided are methods of treating metachromatic leukodystrophy comprising administering to a subject in need of treatment a therapeutically effective amount of recombinant arylsulfatase A enzyme.

The present invention provides, among other things, methods of treatment of Metachromatic Leukodystrophy Disease (MLD) and compositions comprising recombinant arylsulfatase A (ASA) protein using enzyme replacement therapy.