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Aldehydo-l-iduronate

WebApr 4, 2006 · Description. A mild form of mucopolysaccharidosis type 1, a rare lysosomal storage disease characterized by progressive physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate. Patients with MPS1S may have little or no neurological involvement, normal stature and life span, but present development of joints ... WebMPS-II (Hunter Syndrome) This syndrome results from a deficiency of α- l-iduronate-2-sulfatase, an enzyme that hydrolyses sulfated iduronic acid residues in heparan sulfate and dermatan sulfate. Dermatan sulfate and heparan sulfate accumulate in the lysosomes of affected cells. About 370 mutations of the iduronate-2-sulfatase gene, on ...

Aldehyde - Wikipedia

WebOct 2, 2024 · Iduronate 2-sulfatase is a sulfatase enzyme that catalyze the hydrolysis of sulfate ester bonds from a wide variety of substrates, including steroids, carbohydrates and proteins. Iduronate 2-sulfatase is required for the lysosomal degradation of heparan sulfate and dermatan sulfate. Mutations in the iduronate 2-sulfatase gene that result in ... WebThe present invention relates to the production of polysaccharides containing the 1- iduronate subunit, particularly but not exclusively to heparin-type polysaccharides, and also to new... irish literary movement https://round1creative.com

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WebMar 22, 2024 · 英文名称： Active Lysyl Oxidase (LOX) Protein-Lysine 6-Oxidase. 型号： GOY-01D3123 酶与激酶. 物种： Homo sapiens (Human，人) 缓冲液成份： 20mM Tris, 150mM NaCl缓冲液（pH8.0, 含有1mM EDTA, 1mM DTT, 0.01% SKL, 5% Trehalose和Proclin300） 性状：冻干粉. 纯度： > 90% 等电点： 6.2 应用： … WebJan 6, 2013 · Functions. iduronate-2-sulfatase activity / metal ion binding. Processes. chondroitin sulfate catabolic process / glycosaminoglycan catabolic process. Components. lysosomal lumen. General Function. Required for the lysosomal degradation of heparan sulfate and dermatan sulfate. Specific Function. WebAldehydo-L-iduronic acid is the open-chain aldehydo-form of L-iduronic acid. It is a conjugate acid of an aldehydo-L-iduronate. SMILES : C(=O)C(C(C(C(C(=O)O)O)O)O)O … irish liverpool fans

Iduronic acid - Wikipedia

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Aldehydo-l-iduronate

Iduronate 2 Sulfatase - an overview ScienceDirect Topics

http://amigo.geneontology.org/amigo/term/CHEBI:28481

Aldehydo-l-iduronate

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WebLinkto all genes and gene productsannotated to aldehydo-L-iduronic acid (excluding "regulates"). Linkto all direct and indirect annotationsto aldehydo-L-iduronic acid (excluding "regulates"). Linkto all direct and indirect annotations download(limited to first 10,000) for aldehydo-L-iduronic acid (excluding "regulates"). Include "regulates" WebThe meaning of ALDEHYDE is acetaldehyde; broadly : any of a class of highly reactive organic compounds that are analogous to acetaldehyde and characterized by a carbonyl …

WebAn iduronate that is the conjugate base of aldehydo-L-iduronic acid, obtained by deprotonation of the carboxy group; major species at pH 7.3. ChEBI CHEBI:21338 … WebDefinition. An iduronate that is the conjugate base of aldehydo-L-iduronic acid, obtained by deprotonation of the carboxy group; major species at pH 7.3. Stars. This entity has been manually annotated by the ChEBI Team.

WebAldurazyme is indicated in the EU for long-term enzyme replacement therapy in patients with a confirmed diagnosis of mucopolysaccharidosis I (MPS I; alpha-L-iduronidase … WebIduronate-2-sulfatase is an exo -sulfatase that hydrolyzes the C2-sulfate ester bond from nonreducing terminal α-L-iduronic acid residues in HS ( Fig. 1 ), as well as DS. This enzyme has been purified 500,000-fold to homogeneity from human liver ( 73 ).

WebFeb 4, 2024 · Mucopolysaccharidoses are extremely rare diseases that are frequently presenting with structural heart problems of the aortic and mitral valve in combination with myocardial dysfunction. In a substantial proportion, this leads to heart failure and is a leading cause of death in these patients. As this glycosaminoglycan degradation defect is …

WebMPS II (Hunter syndrome) is an X-linked disorder caused by a deficiency of the catalytic enzyme iduronate-2-sulfatase causing characteristic facial features, severe restrictive … irish livestock trucksWebAldehydo-L-iduronate: Spectrum type: 13 C NMR Spectrum (1D, 200 MHz, D 2 O, predicted) Spectrum View. Spectra Viewer Instructions... Experimental Conditions. Solvent: D 2 O: Nucleus: 13 C: Frequency: 200 MHz: Documentation. Document Description Download File Size; Peak List (TXT) Download file: 266 Bytes: Peak Assignments (TXT) ... irish load line certificateWebAldehydo-L-iduronate: Spectrum type: 13 C NMR Spectrum (1D, 100 MHz, D 2 O, predicted) Spectrum View. Spectra Viewer Instructions... Experimental Conditions. … irish liverpool goalkeeperWebaldehyde, any of a class of organic compounds in which a carbon atom shares a double bond with an oxygen atom, a single bond with a hydrogen atom, and a single bond with … irish living room decorWebUses of aldehydes. Hundreds of individual aldehydes are used by chemists daily to synthesize other compounds, but they are less important in industrial synthesis (that is, … irish local development network ildnWebReactome is pathway database which provides intuitive bioinformatics tools for the visualisation, interpretation and analysis of pathway knowledge. port analyzer toolWebα-L-iduronate (CHEBI:193037) is a iduronate (CHEBI:33820) aldehydo-L-iduronate (CHEBI:21338) is a iduronate (CHEBI:33820) iduronic acid (CHEBI:24769) is conjugate … irish liverpool striker