Abstract: A novel protein profiling method of testing for Lysosomal Storage Diseases (“LSD”) using discovered normalized lysosomal fingerprint patterns. The fingerprint patterns reveal the health of lysosomal organelles, specific LSD, and clinical severity. Multiplexing bead technology for simultaneous screening of multiple LSD and normalizing measured enzyme activity or protein levels against other lysosomal proteins, enzymes, or enzyme activities. Compounds, reagents, and methods for identifying and quantifying multiple target enzymes and proteins.

Abstract: A novel protein profiling method of testing for Lysosomal Storage Diseases (“LSD”) using discovered normalized lysosomal fingerprint patterns. The fingerprint patterns reveal the health of lysosomal organelles, specific LSD, and clinical severity. Multiplexing bead technology for simultaneous screening of multiple LSD and normalizing measured enzyme activity or protein levels against other lysosomal proteins, enzymes, or enzyme activities. Compounds, reagents, and methods for identifying and quantifying multiple target enzymes and proteins.

Abstract: A novel protein profiling method of testing for Lysosomal Storage Diseases (“LSD”) using discovered normalized lysosomal fingerprint patterns. The fingerprint patterns reveal the health of lysosomal organelles, specific LSD, and clinical severity Multiplexing bead technology for simultaneous screening of multiple LSD and normalizing measured enzyme activity or protein levels against other lysosomal proteins, enzymes, or enzyme activities. Compounds, reagents, and methods for identifying and quantifying multiple target enzymes and proteins.

Abstract: Multiplexing bead technology is used for simultaneous screening of multiple LSD and normalizing measured enzyme activity or protein levels against other lysosomal proteins, enzymes, or enzyme activities. Diagnostic compositions include microspheres conjugated to purified antibodies that specifically bind LSD target antigens: saposin, LAMP-1, ?-iduronidase, ?-glucosidase, ?-glucosidase, 2-sulphatase, 4-sulphatase, ?-galactosidase, sphingomyelinase, 3-sulphatase or sulphamidase. The target antigens are naturally present in biological fluids or tissues of either LSD or non-LSD patients.

Abstract: The present invention relates generally to mammalian ?-N-acetyglucosaminidase and to genetic sequences encoding same and to their use in the investigation, diagnosis and treatment of subjects suspected of or suffering from ?-N-acetyglucosaminidase deficiency.

Abstract: The invention provides methods of diagnosing or monitoring lysosomal storage disorders based on detecting levels of saposins, LAMPs and/or ?-glucosidase in patient sample. Elevated levels of saposins and/or LAMPs are indicative of a disorder. Elevated levels of ? glucosidase are indicative of some types of lysosomal storage disorders and decreased levels of ? glucosidase are indicative of other types of lysosomal storage disorder. In some methods, the profile of elevation of different saposins, LAMPs and ? glucosidase allows distinction between different types of lysosomal storage disorder.

Abstract: Methods for assaying a lysosomal enzyme activity present in a blood sample obtained from a patient. The method combines the blood or plasma sample in a buffer with at least one binding reagent capable of reacting with alpha-glucosidase present in the blood sample to form an enzyme reagent complex. The lysosomal enzyme activity present in the blood sample is then determined from the enzyme reagent complex formed and compared to a mean level of alpha-glucosidase in a control population of individuals not having a lysosomal storage disease.

Abstract: A method of analyzing and sequencing saccharide material composed of saccharide chains. The saccharide chains are end referenced, e.g., by labeling or tagging at their reducing ends, and the saccharide material is subjected to a controlled partial depolymerisation using a selection scission reagent, for example, low pH nitrous acid, which cleaves internal glycosidic linkages in accordance with known linkage specificity so as to produce a mixed set of saccharide chain fragments having different lengths ranging throughout the full spectrum of possible lengths for the particular glycosidic linkage specificity of the selective scission reagent employed. Samples of the mixed set of saccharide chain fragments are then treated with selected exoenzymes including exoglycosidases that cleave only particular glycosidic linkages at the non-reducing end of saccharide chains. These exoenzymes are applied to the samples either singly or in combination in accordance with a predetermined strategy.

Abstract: The present invention provides a highly glycosylated iduronate-2-sulfatase enzyme comprising an iduronate-2-sulfatase polypeptide with at least 5 kilodalton (kDa) more sugar than iduronate-2-sulfatase purified from a natural source, e.g. human liver. The present invention also provides an enzymatically active polypeptide fragment or variant of such a highly glycosylated iduronate-2-sulfatase. The present invention further provides an isolated nucleic acid encoding iduronate-2-sulfatase, as well as an expression vector, a host cell and a method for producing the present highly glycosylated iduronate-2-sulfatase enzyme.

Abstract: An efficient architecture for an interpolator (100) disposed to process oversampled data is disclosed herein. The interpolator (100) includes an input divider circuit (104) configured to receive an input data word over an input data line. A register (108) is provided for latching the divided input data word from the divider (104). The divided input data word is added within a summer (112) to a latched divided data word from the register, thereby forming a summed data word. A multiplexer (116) produces an interpolated output by multiplexing the summed data word with an input data word. In a preferred implementation, the register (108) is latched at a first clock rate, and the multiplexer (116) is clocked at twice the first clock rate. The efficient filter architecture allows interpolation to be performed in the absence of multipliers, and in a manner using filter coefficients equivalent to powers of two.

Abstract: The present invention relates generally to &agr;-L-iduronidase and to genetic sequences encoding same. More particularly, the present invention provides an isolated nucleic acid molecule comprising a sequence of nucleotides which encodes or are complementary to a sequence which encodes a mammalian &agr;-L-iduronidase or fragment or derivative thereof and to the recombinant enzyme encoded thereby. These molecules are useful in the investigation, diagnosis and treatment of subjects suspected of or suffering from &agr;-L-iduronidase deficiency.

Abstract: The present invention relates generally to mammalian sulphamidase and to genetic sequences encoding same and to the use of these in the investigation, diagnosis and treatment of subjects suspected of or suffering from sulphamidase deficiency.

Abstract: The present invention relates generally to mammalian sulphamidase and to genetic sequences encoding same and to the use of these in the investigation, diagnosis and treatment of subjects suspected of or suffering from sulphamidase deficiency.

Abstract: The present invention relates generally to mammalian sulphamidase and to genetic sequences encoding same and to the use of these in the investigation, diagnosis ad treatment of subjects suspected of or suffering from sulphamidase deficiency.

Abstract: The present invention relates generally to mammalian sulphamidase and to genetic sequences encoding same and to the use of these in the investigation, diagnosis and treatment of subjects suspected of or suffering from sulphamidase deficiency.

Abstract: The present invention relates generally to mammalian &agr;-N-acetyglucosaminidase and to genetic sequences encoding same and to their use in the investigation, diagnosis and treatment of subjects suspected of or suffering from &agr;-N-acetylglucosaminidase deficiency.

Abstract: The present invention relates generally to &agr;-L-iduronidase and to genetic sequences encoding same. More particularly, the present invention provides an isolated nucleic acid molecule comprising a sequence of nucleotides which encodes or are complementary to a sequence which encodes a mammalian &agr;-L-iduronidase or fragment or derivative thereof and to the recombinant enzyme encoded thereby. These molecules are useful in the investigation, diagnosis and treatment of subjects suspected of or suffering from &agr;-L-iduronidase deficiency.

Abstract: The present invention relates generally to mammalian sulphamidase and to genetic sequences encoding same and to the use of these in the investigation, diagnosis and treatment of subjects suspected of or suffering from sulphamidase deficiency.

Abstract: The present invention provides a highly glycosylated iduronate-2-sulfatase enzyme comprising an iduronate-2-sulfatase polypeptide with at least 5 kilodalton (kDa) more sugar than iduronate-2-sulfatase purified from a natural source, e.g. human liver. The present invention also provides an enzymatically active polypeptide fragment or variant of such a highly glycosylated iduronate-2-sulfatase. The present invention further provides an isolated nucleic acid encoding iduronate-2-sulfatase, as well as an expression vector, a host cell and a method for producing the present highly glycosylated iduronate-2-sulfatase enzyme.

Abstract: The present invention relates generally to .alpha.-L-iduronidase and to genetic sequences encoding same. More particularly, the present invention provides an isolated nucleic acid molecule comprising a sequence of nucleotides which encodes or are complementary to a sequence which encodes a mammalian .alpha.-L-iduronidase or fragment or derivative thereof and to the recombinant enzyme encoded thereby. These molecules are useful in the investigation, diagnosis and treatment of subjects suspected of or suffering from .alpha.-L-iduronidase deficiency.