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  • noun

Words related to lysosome

an organelle found in the cytoplasm of most cells (especially in leukocytes and liver and kidney cells)

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Without these important recognition markers, lysosomal enzymes are not transported to the lysosomes; instead, they leak out of cells, and the serum levels of these enzymes are higher than normal.
We have shown that tandem mass spectrometry (MS/MS) can be used together with designed enzyme substrates to quantify the rates of lysosomal enzymes in dried blood spots (DBS) from newborn screening cards (3-8).
Washington, Nov 10 (ANI): For the first time, researchers from Cincinnati Children's Hospital Medical Center have shown how developing red blood cells could be used to produce lysosomal enzymes.
Large Scale Biology Corporation (Vacaville, CA) has patented a method of production of enzymatically active recombinant human and animal lysosomal enzymes involving construction and expression of recombinant expression constructs comprising coding sequences of human or animal lysosomal enzymes in a plant expression system.
A property of HuCNS-SC is that they spread throughout the brain and produce both of the lysosomal enzymes missing in both subtypes of Batten disease being studied in the proposed clinical trial.
A chromatogram that includes the retention and peaks for substrates, products, and internal standards for all 6 lysosomal enzymes is shown in Fig.
In two sub-types of the NCLs - infantile and late infantile or, more technically, CLN1 and CLN2 - normally secreted housekeeping lysosomal enzymes are either defective or missing altogether, as a result of gene mutations.
Our findings demonstrate a unique and somewhat surprising delivery pathway for lysosomal enzymes," said Dao Pan, PhD, corresponding author and researcher in the Division of Experimental Hematology and Cancer Biology at Cincinnati Children's.
Assuming approval, the trial will investigate the safety of transplanting the Company's patented human neural stem cells (HuCNS-SC) in neuronal ceroid lipofuscinoses patients, and determine if the transplanted cells secrete the missing lysosomal enzymes in the brain and alter some characteristics and clinical course of the disease.
We set out to optimize our ESIMS/MS assays by exploring buffer conditions that would allow multiple lysosomal enzymes to be assayed with a single DBS punch in a single assay well, plus a sample workup that no longer required solid-phase extraction.
Assuming approval, we will investigate the safety of transplanting our patented human neural stem cells, HuCNS-SC, in patients, and determine if the transplanted cells secrete the missing lysosomal enzymes in the brain and alter some characteristics and clinical course of the disease.
In addition, preliminary studies indicate that GILT-tagged proteins actually induce more M6P receptor to appear on the cell surface compared to conventional replacement lysosomal enzymes, which may be particularly important for tissues that do not normally express high levels of M6P receptor, like skeletal muscle.
Recombinant DNA techniques have allowed high-throughput production of some lysosomal enzymes.
BioMarin intends to use Synapse's proprietary p97 technology to treat disorders of the brain by delivering lysosomal enzymes and other therapeutics across the blood-brain barrier by means of traditional intravenous injections.
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