An enzyme that catalyzes the hydrolysis of an alpha L-fucoside to yield an alcohol and L-fucose. Deficiency of this enzyme can cause FUCOSIDOSIS. EC 3.2.1.51.
An autosomal recessive lysosomal storage disease caused by a deficiency of ALPHA-L-FUCOSIDASE activity resulting in an accumulation of fucose containing SPHINGOLIPIDS; GLYCOPROTEINS, and mucopolysaccharides (GLYCOSAMINOGLYCANS) in lysosomes. The infantile form (type I) features psychomotor deterioration, MUSCLE SPASTICITY, coarse facial features, growth retardation, skeletal abnormalities, visceromegaly, SEIZURES, recurrent infections, and MACROGLOSSIA, with death occurring in the first decade of life. Juvenile fucosidosis (type II) is the more common variant and features a slowly progressive decline in neurologic function and angiokeratoma corporis diffusum. Type II survival may be through the fourth decade of life. (From Menkes, Textbook of Child Neurology, 5th ed, p87; Am J Med Genet 1991 Jan;38(1):111-31)
An integrin heterodimer widely expressed on cells of hematopoietic origin. CD11A ANTIGEN comprises the alpha chain and the CD18 antigen (ANTIGENS, CD18) the beta chain. Lymphocyte function-associated antigen-1 is a major receptor of T-CELLS; B-CELLS; and GRANULOCYTES. It mediates the leukocyte adhesion reactions underlying cytolytic conjugate formation, helper T-cell interactions, and antibody-dependent killing by NATURAL KILLER CELLS and granulocytes. Intracellular adhesion molecule-1 has been defined as a ligand for lymphocyte function-associated antigen-1.
Cell-surface glycoprotein beta-chains that are non-covalently linked to specific alpha-chains of the CD11 family of leukocyte-adhesion molecules (RECEPTORS, LEUKOCYTE-ADHESION). A defect in the gene encoding CD18 causes LEUKOCYTE-ADHESION DEFICIENCY SYNDROME.
A family of transmembrane glycoproteins (MEMBRANE GLYCOPROTEINS) consisting of noncovalent heterodimers. They interact with a wide variety of ligands including EXTRACELLULAR MATRIX PROTEINS; COMPLEMENT, and other cells, while their intracellular domains interact with the CYTOSKELETON. The integrins consist of at least three identified families: the cytoadhesin receptors(RECEPTORS, CYTOADHESIN), the leukocyte adhesion receptors (RECEPTORS, LEUKOCYTE ADHESION), and the VERY LATE ANTIGEN RECEPTORS. Each family contains a common beta-subunit (INTEGRIN BETA CHAINS) combined with one or more distinct alpha-subunits (INTEGRIN ALPHA CHAINS). These receptors participate in cell-matrix and cell-cell adhesion in many physiologically important processes, including embryological development; HEMOSTASIS; THROMBOSIS; WOUND HEALING; immune and nonimmune defense mechanisms; and oncogenic transformation.
Integrin alpha4beta1 is a FIBRONECTIN and VCAM-1 receptor present on LYMPHOCYTES; MONOCYTES; EOSINOPHILS; NK CELLS and thymocytes. It is involved in both cell-cell and cell- EXTRACELLULAR MATRIX adhesion and plays a role in INFLAMMATION, hematopoietic cell homing and immune function, and has been implicated in skeletal MYOGENESIS; NEURAL CREST migration and proliferation, lymphocyte maturation and morphogenesis of the PLACENTA and HEART.
Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.
Adherence of cells to surfaces or to other cells.
The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining PROTEIN CONFORMATION.
The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.
Plasma glycoprotein member of the serpin superfamily which inhibits TRYPSIN; NEUTROPHIL ELASTASE; and other PROTEOLYTIC ENZYMES.

Endometrial lysosomal enzyme activity in normal cycling endometrium. (1/168)

The objective of this study was to evaluate the possible role of four lysosomal enzymes in endometrial function and remodelling during the normal menstrual cycle by fluorimetric measurement (acid phosphatase, N-acetyl-beta-D-glucosaminidase, alpha-L-fucosidase and alpha-D-mannosidase). A prospective study was conducted of 45 endometrial biopsies obtained from women with normal menstrual cycles. Activity of all four enzymes was identified in human endometrium. Activity of acid phosphatase and N-acetyl-beta-D-glucosaminidase was relatively high, whilst that of alpha-L-fucosidase and alpha-D-mannosidase was low. There was no significant change in the activity of any of the four enzymes from the proliferative to the secretory phase of the cycle. This study suggests that the activity of these enzymes remains constant throughout a major portion of the normal cycle.  (+info)

Characterization of human semen alpha-L-fucosidases. (2/168)

Human semen contains a large amount of alpha-L-fucosidase activity, the great majority of which is found in the seminal fluid. Immunocytochemical studies indicate that a small amount of semen fucosidase activity is present on the sperm plasma membrane, primarily in the posterior head region. Subcellular fractionation studies also indicate that sperm alpha-L-fucosidase is present in the plasma membrane-enriched fraction. Comparative characterization of human seminal fluid and sperm alpha-L-fucosidases indicates that seminal fluid alpha-L-fucosidase has a broad pH optimum curve with a number of near-equal maxima between pH 4.8 and 7.0 while sperm fucosidase has a major optimum between pH 3.4 and 4.0. Isoelectric focusing indicates that seminal fluid alpha-L-fucosidase contains three to six isoforms with isoelectric points (pI) of 5-7 while sperm fucosidase contains two distinct isoforms with pI values of 5. 2 +/- 0.2 and 7.0 +/- 0.2. Western blotting indicates that seminal fluid fucosidase contains a major protein band with a molecular mass ratio (M(r)) of approximately 56 kDa while sperm fucosidase contains a major protein band of approximately 51 kDa. The overall results indicate the presence of a low-abundance, plasma membrane-associated human sperm alpha-L-fucosidase, which is different in its properties from human seminal fluid alpha-L-fucosidase(s), and whose function is not yet known.  (+info)

Fucose in alpha(1-6)-linkage regulates proliferation and histogenesis in reaggregated retinal spheroids of the chick embryo. (3/168)

We have used the lectin from Aleuria aurantia (AAL) which is highly specific for alpha(1-6)-linked fucose, to examine its effect on chicken retinogenesis in a reaggregation culture system. When dispersed cells of the embryonic chick retina are reaggregated to form histotypic retinospheroids, AAL elicits strong inhibition of spheroid growth. The action of AAL is specific, since its effect is dose-dependent, saturable, and inhibited by an excess of fucose. Fucosidase treatment entirely abolishes reaggregation. In contrast, Anguilla anguilla agglutinin (AAA) binding to fucose in alpha(1-2)-linkage does not show any effects. Incubation with CAB4-a specific monoclonal antibody for fucose in alpha(1-6)-linkage-reduces spheroid size and shape. AAL does not much affect primary aggregation, but rather subsequent processes of cell proliferation and histogenesis. In particular, AAL inhibits uptake of bromo-desoxyuridine (BrdU), most efficiently so during days in vitro 2 (div2) and div3. As a consequence, the histological differentiation is entirely disturbed, as evidenced by vimentin immunostaining; particularly, rosettes are not forming and the radial glia scaffold is disorganized. We conclude that glycoproteins exhibiting fucose in alpha(1-6)-linkage may play major roles in early processes of retinal tissue formation.  (+info)

Glycosylation alterations of cells in late phase apoptosis from colon carcinomas. (4/168)

Comparisons of carbohydrate profiles between control and apoptotic colon carcinoma cells were performed by flow cytometry using a set of lectins and anti-carbohydrate antibodies. The six cell lines analyzed presented distinct carbohydrate profiles before induction of apoptosis. PHA-L and MAA binding decreased after induction of apoptosis by UV-treatment. In contrast an increase of PNA binding was observed after induction of apoptosis, except on SW-48 cells for which a decrease occurred. A decrease of SNA binding was observed after induction of apoptosis from strongly positive control cell lines, whereas it increased on weakly positive ones. All the blood group related antigens A, H, Lewis a, Lewis x, Lewis b, and Lewis y, had their expression strongly diminished on apoptotic cells. These changes occurred irrespective of the mode of apoptosis induction since similar results were obtained after UV, TNFalpha, or anti-Fas treatment. Fucosyltransferases activities were also decreased after apoptosis induction, except for alpha1,3fucosyltransferase in anti-Fas treated HT-29 cells, where it was strongly augmented. This could be attributed to the IFNgamma preteatment required to induce Fas expression on these cells. Fucosidase activity decreased after induction of apoptosis suggesting that it was not responsible for the loss of fucosylated structures. In the rat PRO cell line, H blood group antigens are mainly carried by a high molecular weight variant of CD44. It could be shown that the loss of H antigen after induction of apoptosis correlated with a loss of the carrier glycoprotein.  (+info)

Terminal glycosylation in cystic fibrosis. (5/168)

Cystic fibrosis (CF) is a common genetic disease for which the gene was identified within the last decade. Pulmonary disease predominates in this ultimately fatal disease and current therapy only slows the progression. CF transmembrane regulator (CFTR), the gene product, is an integral membrane glycoprotein that normally functions as a chloride channel in epithelial cells. The most common mutation, deltaF508, results in mislocalization and altered glycosylation of CFTR. Altered fucosylation and sialylation are hallmarks of both membrane and secreted glycoproteins in CF and the focus here is on these investigations. Oligosaccharides from CF membrane glycoproteins have the Lewis x, selectin ligand in terminal positions. In addition, two major bacterial pathogens in CF, Pseudomonas aeruginosa and Haemophilus influenzae, have binding proteins, which recognize fucose in alpha1,3 linkage and asialoglycoconjugates. We speculate that the altered terminal glycosylation of airway epithelial glycoproteins in CF contributes to the chronic infection and robust inflammatory response in the CF lung. Understanding the effects of mutant CFTR on glycosylation may provide further insight into the regulation of glycoconjugate processing as well as therapy for CF.  (+info)

Prediction of the development of hepato-cellular-carcinoma in patients with liver cirrhosis by the serial determinations of serum alpha-L-fucosidase activity. (6/168)

OBJECTIVE: Evaluation of the usefulness of the serial determinations of serum alpha-L-fucosidase (AFU) activity for prediction of the development of hepatocellular carcinoma (HCC) was performed. METHODS AND PATIENTS: Serum AFU activity was determined monthly for 42 months in 73 patients with liver cirrhosis (LC). RESULTS: HCC was diagnosed in 27 patients by means of ultrasonography during this observation period. In 23 (85%) of the 27 patients, serum AFU activity was found to exceed 700 nmole/ml/h during the LC stage. HCC developed within a few years in 23 (82%) of 28 LC patients with AFU activity exceeding 700 nmole/ml/h, in contrast, it developed in only 4 (9%) of 45 LC patients with AFU activity below 700 nmole/ml/h. AFU activity was already elevated in 23 (85%) of 27 patients at least 6 months before the detection of HCC by ultrasonography. CONCLUSION: It is conceivable that the development of HCC can be predicted by means of serial determinations of serum AFU activity in patients with LC.  (+info)

Endometrial lysosomal enzyme activity in ovulatory dysfunctional uterine bleeding, IUCD users and post-partum women. (7/168)

The aim of this study was to evaluate the role of lysosomal enzymes in excessively heavy menstruation by comparing women with menorrhagia due to dysfunctional bleeding or intrauterine contraceptive device (IUCD) use with those with normal menstrual periods or with amenorrhoea associated with breastfeeding. This was a prospective cohort investigation of the activity of four endometrial lysosomal enzymes in three contrasting groups: (i) women with ovulatory dysfunctional uterine bleeding and users of intrauterine contraceptive devices; (ii) breastfeeding post-partum women in whom there are long periods of amenorrhoea, particularly in the early months post-partum; and (iii) normal cycling women. It was found that the total activity of lysosomal enzymes, particularly acid phosphatase and N-acetyl-beta-D-glucosaminidase, was markedly elevated (P < 0.001) in IUCD-exposed endometrium, and endometrium from women with dysfunctional uterine bleeding when compared with endometrium from women with a history of entirely normal menstrual periods or that in post-partum breastfeeding women. The activity of alpha-L-fucosidase was moderately elevated in IUCD users (P < 0.05) and ovulatory dysfunctional uterine bleeding (P < 0.05), whereas alphaD-mannosidase activity was elevated in ovulatory dysfunctional uterine bleeding (P < 0.05), but decreased in IUCD users (P < 0.01). No significant differences were observed in the lysosomal enzyme activities of breastfeeding post-partum women and normal cycling women. These results show that total endometrial tissue activity of four lysosomal enzymes was substantially increased throughout the cycle in most circumstances in women with two different causes for increased menstrual bleeding. This suggests a contributory role to the increased bleeding.  (+info)

Sequence and expression of Thai Rosewood beta-glucosidase/beta-fucosidase, a family 1 glycosyl hydrolase glycoprotein. (8/168)

Dalcochinin-8'-O-beta-glucoside beta-glucosidase (dalcochinase) from the Thai rosewood (Dalbergia cochinchinensis Pierre) has aglycone specificity for isoflavonoids and can hydrolyze both beta-glucosides and beta-fucosides. To determine its structure and evolutionary lineage, the sequence of the enzyme was determined by peptide sequencing followed by PCR cloning. The cDNA included a reading frame coding for 547 amino acids including a 23 amino acid propeptide and a 524 amino acid mature protein. The sequences determined at peptide level were found in the cDNA sequence, indicating the sequence obtained was indeed the dalcochinase enzyme. The mature enzyme is 60% identical to the cyanogenic beta-glucosidase from white clover glycosyl hydrolase family 1, for which an X-ray crystal structure has been solved. Based on this homology, residues which may contribute to the different substrate specificities of the two enzymes were identified. Eight putative glycosylation sites were identified, and one was confirmed to be glycosylated by Edman degradation and mass spectrometry. The protein was expressed as a prepro-alpha-mating factor fusion in Pichia pastoris, and the activity of the secreted enzyme was characterized. The recombinant enzyme and the enzyme purified from seeds showed the same K(m) for pNP-glucoside and pNP-fucoside, had the same ratio of V(max) for these substrates, and similarly hydrolyzed the natural substrate, dalcochinin-8'-beta-glucoside.  (+info)

Définitions de 1 3 alpha l fucosidase, synonymes, antonymes, dérivés de 1 3 alpha l fucosidase, dictionnaire analogique de 1 3 alpha l fucosidase (anglais)
Alpha-l-fucosidase (AFU) plays vital roles in some physiological processes that closely relate to several diseases. Herein, a ratiometric fluorescent probe for the alpha-l-fucosidase (AFU) assay based on a single fluorophore has been developed by functionalizing a new fluorophore platform (6-bromo-anthracene MCF Editors Recommendation
While the catalytic nucleophile in the configuration-retaining alpha-L-fucosidases from family GH29 is fully conserved with respect to sequence, there is no fully sequence-conserved acid/base residue candidate across the family. X-ray crystallographic studies and kinetic characterizations have allowed the identification of this residue in a few cases, and a recent combination of phylogenetic tree analyses with substrate specificity data has allowed the division of GH29 enzymes into two subfamilies, A and B, allowing the probable assignment of these residues. Here, we perform detailed kinetic and mechanistic characterizations of the corresponding alanine mutants and other candidates. Through comparison of kinetic parameters obtained for the hydrolysis of fucosyl substrates with activated leaving groups by these mutants with those of the corresponding wild-type enzymes, in conjunction with the demonstration of azide rescue, we largely confirm the acid/base residue predictions for the GH29 ...
BioAssay record AID 36841 submitted by ChEMBL: Inhibition of the enzyme alpha-L-fucosidase from bovine epididymis was determined at 1 mM concentration of the compound; No inhibition.
K01186 NEU1; sialidase-1 [EC:3.2.1.18] K01186 NEU1; sialidase-1 [EC:3.2.1.18] K01190 lacZ; beta-galactosidase [EC:3.2.1.23] K12373 HEXA_B; hexosaminidase [EC:3.2.1.52] K12373 HEXA_B; hexosaminidase [EC:3.2.1.52] K01191 MAN2C1; alpha-mannosidase [EC:3.2.1.24] K01227 E3.2.1.96; mannosyl-glycoprotein endo-beta-N-acetylglucosaminidase [EC:3.2.1.96] K01206 FUCA; alpha-L-fucosidase [EC:3.2.1.51] K15923 AXY8; alpha-L-fucosidase 2 [EC:3.2.1.51 ...
TY - JOUR. T1 - Synthesis and evaluation of a 5-Membered Isoiminosugar as Glycosidase Inhibitor. AU - Lundt, Inge. AU - Godskesen, Michael Anders. PY - 1998. Y1 - 1998. N2 - (3R, 4R)-4-hydroxy-3-hydroxymethylpyrrolidine (6) was prepared from D-xylose and found to be a weak inhibitor of alpha-D-, beta-D-glucosidase and alpha-L-fucosidase. (C) 1998 Published by Elsevier Science Ltd. All rights reserved. AB - (3R, 4R)-4-hydroxy-3-hydroxymethylpyrrolidine (6) was prepared from D-xylose and found to be a weak inhibitor of alpha-D-, beta-D-glucosidase and alpha-L-fucosidase. (C) 1998 Published by Elsevier Science Ltd. All rights reserved. U2 - 10.1016/S0040-4039(98)01157-5. DO - 10.1016/S0040-4039(98)01157-5. M3 - Journal article. VL - 39. SP - 5841. EP - 5844. JO - Tetrahedron Letters. JF - Tetrahedron Letters. SN - 0040-4039. IS - 32. ER - ...
Glycosylation drives critical processes important for mammalian cell-cell and cell-matrix interactions. Alpha-l-fucose (α-l-f) is a key monosaccharide component of oligosaccharides that has been found to be overexpressed during tumor progression. Modification of cell surface fucosylation, we hypothesized, alters tumor cell phenotype and function at the end of the neoplastic progression cascade including tumor invasion. Alpha-l-fucosidase (α-l-fase) is a glycosidase that specifically removes (α-l-f) from oligosaccharide sites. We first verified the effectiveness of the α-l-fase to specifically decrease the level of α-l-f on the cell surface of several human breast cancer cell lines and also examined the recovery time for these cells to repopulate their surfaces. To investigate the potential effect of defucosylation on tumor functions, we studied the proliferation, and invasion in vitro of human breast cancer MDA-MB-231 cells as the representative cell model. We further examined several ...
Math o ganser syn cychwyn yn yr afu yw canser yr afu, a elwir hefyd yn ganser hepatig a chanser hepatig cynradd.[1] Mae canserau sydd wedi lledaeni ir afu o lefydd eraill yn y corff (metastasis yr afu) llawer mwy cyffredin na chyflyrau syn cychwyn yn yr afu.[2] Gall canser yr afu achosi symptomau megis ymdeimlad o boen neu lwmp gweledol islawr gawell asennau ar yr ochr dde, chwyddo yn yr abdomen, croen melyn, cleisio hawdd, colli pwysau a gwendid cyffredinol. Achosir y rhan fwyaf o gyflyrau canser yr afu gan sirosis o ganlyniad i hepatitis B, hepatitis C, neu alcohol.[3] Ymhlith yr achosion eraill y mae afflatocsin, afiechyd afu brasterog di-alcohol, a llyngyr yr afu. Y mathau mwyaf cyffredin or cyflwr yw carsinoma hepatogellol neu HCC (80% o achosion), a cholangiocarcinoma. Ceir rhai llai cyffredin yn ogystal, er enghraifft neoplasm systig mwsinog a neoplasm bustlaidd papilaidd anhydradwyol. Gellir cadarnhau diagnosis drwy brawf gwaed a delweddu meddygol a chanfod tystiolaeth bellach or ...
Complete information for FUCA1P1 gene (Pseudogene), Alpha-L-Fucosidase 1 Pseudogene 1, including: function, proteins, disorders, pathways, orthologs, and expression. GeneCards - The Human Gene Compendium
Fucosidases - Instruments Consumables Reagents Advanced BioMatrix,RANDOX,RANDOX ELISA,Biomedical, biochemical reagents, laboratory supplies, equipment, antibodies, ELISA kits, diagnostic reagents, methods of experimental techniques, general analytical instruments, material testing instruments and equipment, used laboratory equipment, instruments and equipment, life sciences, environmental monitoring equipment , measurement, measuring instruments, rotating wall bioreactor, three-dimensional tissue / stem cell culture system; microcapsule
A series of phenyl-D-thioglucopyranosides were prepared and characterized in order to determine the absolute specificity of the .B-thioglucosidase and B-glucosidese enzyme systems toward the th1oglucosidic bond. The specificity of B-glucosidase of almond emulsin toward oxygen linkage seems to be absolute, since no hydrolysis was observed by this enzyme system toward the phenyl-D-thioglucopyranosides. The P-th1oglucosidase system was found to hydrolyze only two of the thioglucopyranosides. It was apparent that only where the electron-attracting substituents in the phenyl group were strong enough to greatly weaken the strength of the sulfur linkage, as in the case of the nitro group, would there be any hydrolysis. The lack of hydrolysis prevented application of the Hammett equation in testing the quantitative electronic effects of the substitutents on the rate of bond hydrolysis.
Complete information for FUCA1 gene (Protein Coding), Fucosidase, Alpha-L- 1, Tissue, including: function, proteins, disorders, pathways, orthologs, and expression. GeneCards - The Human Gene Compendium
The chemical relationship of the seven forms of human liver α-L fucosidase has been studied by isoelectric focusing of neuraminidase- and sialyltransf
In a previous study, we molecular-characterized a tomato (Solanum lycopersicum) α1, 3/4-fucosidase (α-Fucase Sl-1) encoded in a… Expand ...
endo-β-1,4-xylanase (EC 3.2.1.8); β-glucosidase (3.2.1.21); β-glucuronidase (EC 3.2.1.31); β-xylosidase (EC 3.2.1.37); β-fucosidase (EC 3.2.1.38); glucosylceramidase (EC 3.2.1.45); β-1,6-glucanase (EC 3.2.1.75); glucuronoarabinoxylan endo-β-1,4-xylanase (EC 3.2.1.136); endo-β-1,6-galactanase (EC:3.2.1.164); [reducing end] β-xylosidase (EC 3.2.1.- ...
endo-β-1,4-xylanase (EC 3.2.1.8); β-glucosidase (3.2.1.21); β-glucuronidase (EC 3.2.1.31); β-xylosidase (EC 3.2.1.37); β-fucosidase (EC 3.2.1.38); glucosylceramidase (EC 3.2.1.45); β-1,6-glucanase (EC 3.2.1.75); glucuronoarabinoxylan endo-β-1,4-xylanase (EC 3.2.1.136); endo-β-1,6-galactanase (EC:3.2.1.164); [reducing end] β-xylosidase (EC 3.2.1.- ...
Fast delivery of FUCA2 knockout Human Cell Lines for the study of gene function. Created by CRISPR/Cas9 genome editing. Includes matched wildtype control.
chains in the Genus database with same CATH superfamily 2X9K A; 2WJR A; 2WVP A; 4FQE A; 2WJQ A; 2IWW A; 4CTD A; 2F1C X; 2IWV A; 4PR7 A; 2JQY A; #chains in the Genus database with same CATH topology 3A2S X; 4O4U A; 2M06 A; 3GP6 A; 2R8A A; 2WVP A; 2Y0L A; 3AA0 A; 1ZHX A; 3AA6 A; 5DL6 A; 4GCS A; 3LK2 B; 4O3Y A; 5AFU L; 2IWV A; 3QRC A; 4KR8 A; 5KAF O; 4AFK A; 2WJR A; 3LK2 A; 3VY9 X; 2J4U P; 3SYB A; 3AAE B; 4LSF A; 4CTD A; 1BT9 A; 5AFU K; 3K19 A; 1MM4 A; 2W80 C; 3POU A; 1ORM A; 3EMN X; 1GFM A; 3AAE A; 2AXT O; 4FRX A; 2LFU A; 4AKR B; 4AUI A; 2XE1 A; 3A0B O; 2M07 A; 4O49 A; 4PUX A; 2J1N A; 2N2L A; 4GHB A; 4FZM A; 3DA4 A; 5G26 A; 5T5F A; 4C00 A; 4AKR A; 4K3B A; 2Y2X A; 5E79 O; 5EE2 A; 3POX A; 3UPG A; 4FES A; 3LK4 1; 4RLB A; 2XTQ A; 2R88 A; 4F4B A; 3LK3 A; 2F1V A; 1OPF A; 1ZHZ A; 3PRN A; 4IL6 O; 4E1S A; 2ERV A; 5DL7 A; 3NJT A; 1HXT A; 3DZM A; 1GFO A; 1T16 A; 2MHL A; 2R4N A; 5B66 O; 4XNL A; 5H2F O; 1IZN B; 3NSG A; 3TZG A; 2Y0K A; 2R4L A; 4FT6 A; 1QJP A; 2JMM A; 2LHF A; 3PGU A; 4B61 A; 4JFB A; 2KC0 A; 4FQE ...
chains in the Genus database with same CATH superfamily 4XNL A; 4AZL A; 4AFK A; 5D5D A; 4B61 A; 5IYU A; 3RBH A; 4XNK A; #chains in the Genus database with same CATH topology 2R4O A; 1GFM A; 1YS5 A; 1T16 A; 2XTQ A; 3G7G A; 3LK3 B; 2Y0L A; 1H6S 1; 4FRX A; 5KAF O; 4FQE A; 3TZG A; 5AFU K; 3POX A; 4RL9 A; 1P4T A; 2F1T A; 4U9C A; 2X27 X; 3AA6 A; 2W81 C; 3SYS A; 5GV0 A; 3V8U A; 5DL5 A; 4AYI D; 3WI4 A; 3SZV A; 1BH3 A; 2M07 A; 4FZM A; 3PGU A; 3VE1 A; 4C00 A; 3T24 A; 4GHB A; 5JDP A; 4O3Z A; 2XMX A; 3BRY A; 4D5U A; 4AKR B; 2M06 A; 3A2S X; 4GF4 A; 2KZ7 B; 3DA3 A; 3POQ A; 4FSP A; 2AXT O; 3HOE A; 5FVN A; 3DWN A; 3PF1 A; 3RBH A; 3AA0 B; 4LSE A; 2F1V A; 2XG6 A; 2X9K A; 1Q9F A; 4PR7 A; 4B61 A; 3HW9 A; 4O3W A; 5G39 A; 4JCH A; 2MHL A; 5AFU L; 2W80 C; 2R4P A; 1QJ9 A; 3POR A; 5IYU A; 2KXP B; 1ORM A; 4KR8 A; 4K3C A; 1IZN A; 4O4U A; 5D5D A; 3PRN A; 4AYD C; 4KR4 A; 3VZW X; 5DL7 A; 1GFO A; 3BS0 A; 3LK4 1; 2N2L A; 3NSG A; 4G75 A; 4JFB A; 4LSI A; 4O4X A; 4RLC A; 3T0S A; 3AA7 B; 2KZ7 A; 3VZU X; 4D64 A; 2R4N A; 4AKR A; 3PQU ...
The molecular basis of the deficiency of alpha-L-fucosidase has been investigated in eight patients who had been diagnosed clinically and enzymatically as suffering from the autosomal recessive lysosomal storage disease fucosidosis. None of the patients had a deletion or gross alteration of the alpha-L-fucosidase gene (FUCA1). Single strand conformation polymorphism (SSCP) analysis followed by direct sequencing of amplified exons and flanking regions identified putative disease causing mutations in six of the patients, who had severe forms of the disease and very low residual alpha-L-fucosidase activity and protein. They were a 10 bp deletion in exon 1 (E113fs), a 1 bp deletion at position -2 of intron 2 (S216fs), a g--,a transition at IVS5+1, point mutations W183X and N329Y in exons 3 and 6, respectively, and a compound allele consisting of a point mutation in the signal peptide in exon 1, P5R, and a 1 bp insertion in exon 6 (Y330fs). One patient in whom an SSCP change was not detected had ...
Severe progressive fatal neurological degeneration occurs in fucosidosis, a storage disease. Bone marrow transplantation into affected dogs has shown that haematopoietic stem cells can provide enzyme producing daughter cells to the central nervous system, altering disease course. This makes canine fucosidosis an ideal large animal model for gene therapy. Fucosidosis affected allogeneic or autologous canine marrow was transduced ex vivo by cocultivation, then transplanted into fucosidosis affected dogs conditioned with total lymphoid irradiation. The vectors were Moloney murine leukaemia virus based. Transduction efficiency was increased with multiple cytokines in short term marrow culture. Despite high levels of transduction, proviral sequence was detected 2 months post transplant in only one dog. Early or total graft failure occurred in all transplants. We believe lack of engraftment could be caused by differentiation or change of repopulating ability of marrow cells occurring with multiple ...
The desire for improved methods of biomass conversion into fuels and feedstocks has re-awakened interest in the enzymology of plant cell wall degradation. The complex polysaccharide xyloglucan is abundant in plant matter, where it may account for up to 20% of the total primary cell wall carbohydrates. Despite this, few studies have focused on xyloglucan saccharification, which requires a consortium of enzymes including endo-xyloglucanases, alpha-xylosidases, beta-galactosidases and alpha-L-fucosidases, among others. In the present paper, we show the characterization of Xy131A, a key alpha-xylosidase in xyloglucan utilization by the model Gram-negative soil saprophyte Cellvibrio japonicus. CjXy131A exhibits high regiospecificity for the hydrolysis of XGOs (xylogluco-oligosaccharides), with a particular preference for longer substrates. Crystallographic structures of both the apo enzyme and the trapped covalent 5-fluoro-beta-xylosyl-enzyme intermediate, together with docking studies with the XXXG ...
Fucosidosis is a rare lysosomal storage disorder caused by the inherited deficiency of the lysosomal hydrolase α-L-fucosidase, which leads to an impaired degradation of fucosylated glycoconjugates. Here we report the generation of a fucosidosis mouse model, in which the gene for lysosomal α-L-fucosidase (Fuca1) was disrupted by gene targeting. Homozygous knock-out mice completely lack α-L-fucosidase activity in all tested organs leading to highly elevated amounts of the core-fucosylated glycoasparagine Fuc(α1,6)-GlcNAc(β1-N)-Asn and, to a lesser extent, other fucosylated glycoasparagines, which all were also partially excreted with the urine. Lysosomal storage pathology was observed in many visceral organs like liver, kidney, spleen and bladder as well as in the CNS. On the cellular level storage was characterized by membrane-limited cytoplasmic vacuoles primarily containing water-soluble storage material. In the CNS cellular alterations included enlargement of the lysosomal compartment in ...
Gauthami S. Kondagari, Barbara M. King, Peter C. Thomson, Peter Williamson, Peter R. Clements, Maria Fuller, Kim M. Hemsley, John J. Hopwood, Rosanne M. Taylor ...
Several 2-(aminomethyl)-and 2-(2-aminoethyl)-pyrrolidine-3,4-diol derivatives have been assayed for their inhibitory activities towards glycosidases. Good inhibitors of alpha-mannosidases must have the (2R,3R,4S) configuration and possess 2-(benzylamino)methyl substituents. Stereomers with the (2S,3R,4S) configuration are also competitive inhibitors of alpha-mannosidases, but less potent as they share the configuration of C(1), C(2), C(3) Of beta-D-mannosides rather than that Of alpha-D-mannosides. Interestingly, (2S,3R,4S)-2-{2-[(4-phenyl)phenylamino]ethyl}pyrrolidine-3,4-diol (12g) inhibits several enzymes, for instance alpha-L-fucosidase from bovine epididymis (K-i = 6.5 muM, competitive), alpha-galactosidase from bovine liver (K-i = 5 muM, mixed) and alpha-mannosidase from jack bean (K-i = 102 muM, mixed). Diamines such as (2R,3S,4R)-2-[2-(phenylamino) or 2-(benzylamino)ethyl]pyrrolidine-3,4-diol (ent-1 2a, ent-12b) inhibit P-glucosidase from almonds (K-i = 13-40 muM, competitive). (C) 2003 Elsevier
The Australian Financial Complaints Authority (AFCA) is a non-government dispute resolution organisation providing free, fair and independent help with disputes between consumers and financial providers. It is compulsory for all Australian Financial Services Licence and Australian Credit Licence providers to be members of AFCA. To have a complaint considered by AFCA it has to be within six years after you first became aware of the loss. With an exception of the current one-year window to consider complaints dating back to 1 January 2008. AFCA is only allowed to accept legacy complaints until 30 June 2020 ...
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Recombinant Human FUCA1 protein is an Insect Full length protein 28 to 466 aa range, | 90% purity, | 1.000 Eu/µg endotoxin level and validated in SDS-PAGE.
Expression of FUCA2 (dJ20N2.5, MGC1314) in caudate tissue. Antibody staining with HPA031659, HPA031660 and HPA031661 in immunohistochemistry.
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At least 26 mutations in the FUCA1 gene have been found to cause fucosidosis. Most of these mutations result in an abnormally short, nonfunctional alpha-L-fucosidase enzyme. Without this enzyme, glycolipids and glycoproteins cannot be completely broken down. These partially broken down compounds accumulate in the lysosomes and cause cells throughout the body to malfunction. Brain cells are particularly sensitive to the buildup of glycolipids and glycoproteins, which can result in cell death. Loss of brain cells is thought to cause the neurological symptoms of fucosidosis. Accumulation of glycolipids and glycoproteins also occurs in other organs such as the liver, spleen, skin, heart, pancreas, and kidneys, contributing to the additional symptoms of fucosidosis. ...
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Önenli-Mungan N, Özer G, Altunbaşak Ş, Besley G, Yüksel B, Topaloğlu AK, Soyupak S. Fucosidosis with hypothyroidism: a case report. Turk J Pediatr 2004; 46: 170-173.. Fucosidosis is a rare, autosomal recessive lysosomal storage disorder caused by a severe deficiency of [alpha]-L-fucosidase.. Here we present a 27-month-old male who was referred to us for evaluation of developmental delay, which was first detected at age six months. His past medical history was also remarkable for recurrent pulmonary infections and myoclonic seiures. His family history revealed that he was the first living child from a consanguineous marriage. He had a younger sister who died at five months of age from pneumonia who had facial resemblance to the proband, developmental delay and a congenital heart defect.. Physical examination revealed length: 81 cm (25-50p), weight: 10.2 kg (25-50p), and head circumference: 49 cm (50-75p). He had a coarse face, hepatomegaly and generalized spasticity.. His initial laboratory ...
Background: Some salivary markers of alcohol abuse/dependence have been proposed so far: aminotransferases, gamma-glutamyltransferase, ethanol, ethyl glucuronide, ethyl sulfate, sialic acid, β-hexosaminidase A, oral peroxidase, methanol, diethylene/ethylene glycol, α-amylase, clusterin, haptoglobin, heavy/light chains of immunoglobulins and transferrin. Aim: To investigate the effect of chronic alcohol drinking and smoking on the activity (pKat/ml) and output (pKat/min) of salivary lysosomal exoglycosidases: α-fucosidase (FUC), α-mannosidase (MAN), β-galactosidase (GAL), and β-glucuronidase (GLU), and their applicability as markers of alcohol dependence. Methods: The activity of FUC, MAN, GAL and GLU was measured colorimetrically in the saliva of healthy social drinkers, alcohol-dependent non-smokers and alcohol-dependent smokers. Results: We observed an increased salivary activity of FUC, GAL, GLU and MAN, as well as an increased output of GAL and GLU, in comparison with controls. The ...
Catheter ablation of atrial fibrillation (AFCA) efficacy in patients with impaired left ventricular systolic function improves when performed early in the natural history of atrial fibrillation (AF) and heart failure, according to a study published recently in Circulation: Arrhythmia and Electrophysiology.1 AFCA provides long-term benefits on left ventricular function, significantly reducing the number of patients with severely impaired systolic function, the authors add. ...
Expression of FUCA2 (dJ20N2.5, MGC1314) in endometrium tissue. Antibody staining with HPA031659 and HPA031661 in immunohistochemistry.
A friend of mine raised some concerns on her blog about Bill 106 - The Safer Communities and Neighbourhoods Act, which was recently introduced into the Ontario Legislature as a private members Bill by Ottawa Centre Liberal MPP Yasir Naqvi. This post is an extension of the comments I left as responses to her post.…
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Sperm-associated antigen 8 is a protein that in humans is encoded by the SPAG8 gene. The correlation of anti-sperm antibodies with cases of unexplained infertility implicates a role for these antibodies in blocking fertilization. Improved diagnosis and treatment of immunologic infertility, as well as identification of proteins for targeted contraception, are dependent on the identification and characterization of relevant sperm antigens. The protein encoded by this gene is recognized by sperm agglutinating antibodies from an infertile woman. This protein is localized in germ cells of the testis at all stages of spermatogenesis and is localized to the acrosomal region of mature spermatozoa. Alternatively spliced variants that encode different protein isoforms have been described but the full-length sequences of only two have been determined. GRCh38: Ensembl release 89: ENSG00000137098 - Ensembl, May 2017 GRCm38: Ensembl release 89: ENSMUSG00000066196 - Ensembl, May 2017 Human PubMed Reference:. ...
Domain architecture and assignment details (superfamily, family, region, evalue) for Afu5g13040 from Aspergillus fumigatus Af293. Plus protein sequence and external database links.
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Plasminogen, the proenzyme of the fibrinolytically active enzyme plasmin, is a single chain glycoprotein with a molecular weight of about 90,000-94,000 Da (2). Various isoelectric forms exist and can be separated by means of isoelectric focusing (IEF).
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Toshiba has begun shipping a 14TB helium-filled hard drive to clients, its largest capacity to date and the company's first foray into helium territory. Of course, helium-filled HDDs are not new, and WD already released a 14TB HDD a couple of months ago. However, Toshiba's is the industry's first HDD to pack nine disks inside, and the only 14TB drive to use conventional magnetic recording (CMR) technology.The new drive is part of Toshiba's MG07ACA series. It is available in both 12TB and 14TB capacities, both of which are CMR drives.We have raised the bar with the new MG07ACA Series 9-disk helium-sealed design, said Akitoshi Iwata, Vice President of Storage Products Division, Toshiba Electronic Devices and Storage Corporation. By utilizing an innovative design, we continue to improve the benefits that high-capacity disk storage can deliver to our broad global customer base.There are a couple of things to note about the 14TB model that make it sta
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... may refer to one of two enzymes: Fucosidase Alpha-L-fucosidase This set index page lists enzyme articles ...
1,2-alpha-L-fucosidase 1,3-alpha-L-fucosidase 1,6-alpha-L-fucosidase FUCA2 Endreffy I, Bjørklund G, Szerafin L, Urbina MA, ... In enzymology, an alpha-L-fucosidase (EC 3.2.1.51) is an enzyme that catalyzes the chemical reaction an alpha-L-fucoside + H2O ... The systematic name of this enzyme class is alpha-L-fucoside fucohydrolase. This enzyme is also called alpha-fucosidase. This ... doi: 10.1007/s12026-017-8943-x. Levvy GA, Mcallan A (August 1961). "Mammalian fucosidases. 2. alpha-L-Fucosidase". The ...
In enzymology, a 1,2-alpha-L-fucosidase (EC 3.2.1.63) is an enzyme that catalyzes the chemical reaction methyl-2-alpha-L- ... Other names in common use include almond emulsin fucosidase, and alpha-(1->2)-L-fucosidase. As of late 2007, 4 structures have ... 2-alpha-L-fucosidase". J. Biol. Chem. 245 (2): 299-304. PMID 5460888. Ogata-Arakawa M, Muramatsu T, Kobata A (1977). "alpha-L- ... Purification and properties of alpha-L-fucosidase from Chamelea gallina L". Eur. J. Biochem. 66 (2): 379-87. doi:10.1111/j.1432 ...
In enzymology, a 1,3-alpha-L-fucosidase (EC 3.2.1.111) is an enzyme that catalyzes the chemical reaction of cleaving the 1,3- ... Yoshima H, Takasaki S, Ito-Mega S, Kobata A (1979). "Purification of almond emulsin alpha-L-fucosidase I by affinity ... Ogata-Arakawa M, Muramatsu T, Kobata A (1977). "alpha-L-fucosidases from almond emulsin: characterization of the two enzymes ... The systematic name of this enzyme class is 3-alpha-L-fucosyl-N-acetylglucosaminyl-glycoprotein fucohydrolase. This enzyme is ...
This enzyme is also called alpha-L-fucosidase. Yazawa S, Madiyalakan R, Chawda RP, Matta KL (1986). "alpha-L-fucosidase from ... 6-alpha-L-fucosidase (EC 3.2.1.127) is an enzyme that catalyzes the chemical reaction Hydrolysis of 1,6-linkages between alpha- ... aspergillus niger: demonstration of a novel alpha-L-(1----6)-fucosidase acting on glycopeptides". Biochem. Biophys. Res. Commun ...
Tissue alpha-L-fucosidase is an enzyme that in humans is encoded by the FUCA1 gene. Alpha-Fucosidase is an enzyme that breaks ... "Entrez Gene: FUCA1 fucosidase, alpha-L- 1, tissue". HPRD entry [1] Archived 2004-10-24 at the Wayback Machine Willems PJ, Gatti ... Fukushima H, de Wet JR, O'Brien JS (1985). "Molecular cloning of a cDNA for human alpha-L-fucosidase". Proc. Natl. Acad. Sci. U ... Yang M, Allen H, DiCioccio RA (1993). "A mutation generating a stop codon in the alpha-L-fucosidase gene of a fucosidosis ...
Alpha-L-fucosidase is responsible for hydrolysing the alpha-1,6-linked fucose joined to the reducing-end N-acetylglucosamine of ... Glycoside hydrolase family 29 includes alpha-L-fucosidases, They are lysosomal enzymes responsible for hydrolyzing the alpha-1, ... making alpha-l-fucosidases, the enzymes responsible for their processing, critically important. Deficiency in alpha-l- ... In human sperm, membrane-associated alpha-l-fucosidase is stable for extended periods of time, which is made possible by ...
Plasma alpha-L-fucosidase (see alpha-L-fucosidase) is an enzyme that in humans is encoded by the FUCA2 gene. GRCh38: Ensembl ... "Entrez Gene: FUCA2 fucosidase, alpha-L- 2, plasma". Narahara K, Tsuji K, Yokoyama Y, et al. (1991). "Specification of small ... Eiberg H, Mohr J, Nielsen LS (Oct 1984). "Linkage of plasma alpha-L-fucosidase (FUCA2) and the plasminogen (PLG) system". Clin ... 2001). "Cell surface human alpha-L-fucosidase". Eur. J. Biochem. 268 (11): 3321-31. doi:10.1046/j.1432-1327.2001.02237.x. PMID ...
The gene encoding the alpha-fucosidase, FUCA 1, was found to be located to the short arm of chromosome 1p36 - p34, by Carrit ... If they are present, a skin or blood sample will be taken to test for below-normal amounts of alpha-fucosidase. - Fucosidosis ... The FUCA1 gene provides instructions for making an enzyme called alpha-L-fucosidase. The enzyme plays a role in the breakdown ... The deficiency of the enzyme alpha-L-fucosidase, which is used to metabolize complex compounds in the body (fucose-containing ...
"Alpha fucosidase and beta galactosidase in serum of a Lyme disease patients as a possible marker of accelerated senescence - a ...
Zhang SY, Lin BD, Li BR (2015). "Evaluation of the diagnostic value of alpha-l-fucosidase, alpha-fetoprotein and thymidine ...
"An alpha-L-fucosidase from Penicillium multicolor as a candidate enzyme for the synthesis of alpha (1-->3)-linked fucosyl ... Penicillium multicolor is an anamorph species of the genus Penicillium which produces alpha-L-fucosidase, tilactase, ... Ajisaka, K.; Fujimoto, H.; Miyasato, M. (1998). "An α-l-fucosidase from Penicillium multicolor as a candidate enzyme for the ...
... alpha-L-fucosidase, poly(1,2-alpha-L-fucoside-4-sulfate) glycanohydrolase) is an enzyme with systematic name poly((1->2)-alpha- ... This enzyme catalyses the following chemical reaction Endohydrolysis of (1->2)-alpha-L-fucoside linkages in fucoidan without ...
... alpha-L-fucosidase MeSH D08.811.277.450.410 - galactosidases MeSH D08.811.277.450.410.050 - alpha-galactosidase MeSH D08.811. ... gtp-binding protein alpha subunits MeSH D08.811.277.040.330.300.200.100.100 - gtp-binding protein alpha subunits, g12-g13 MeSH ... gtp-binding protein alpha subunit, gi2 MeSH D08.811.277.040.330.300.200.100.300 - gtp-binding protein alpha subunits, gq-g11 ... steroid 12-alpha-hydroxylase MeSH D08.811.682.690.708.170.915.737 - steroid 16-alpha-hydroxylase MeSH D08.811.682.690.708.170. ...
... alpha/beta-galactosidase, β-glucuronidase, α-fucosidase, α-mannosidase, and trypsin. Important dual virulence factors found in ...
... beta-D-glucosides and alpha-L-arabinosides. Chinchetru MA, Cabezas JA, Calvo P (1983). "Characterization and kinetics of beta-D ... Beta-D-fucosidase (EC 3.2.1.38, beta-fucosidase) is an enzyme with systematic name beta-D-fucoside fucohydrolase. This enzyme ... Beta-D-fucosidase at the US National Library of Medicine Medical Subject Headings (MeSH) Portal: Biology (EC 3.2.1). ... Wiederschain GY, Prokopenkov AA (October 1973). "Beta-D-galactosidase and beta-D-fucosidase of pig kidney". Archives of ...
CRYSTAL STRUCTURE OF THERMOTOGA MARITIMA ALPHA-FUCOSIDASE IN COMPLEX WITH A MECHANISM BASED INHIBITOR ... PUTATIVE ALPHA-L-FUCOSIDASE. A, B. 449. Thermotoga maritima MSB8. Mutation(s): 0 Gene Names: TM_0306. EC: 3.2.1.51. ... and alpha-l-fucosidases, the enzymes responsible for their processing, are therefore of crucial importance. Deficiency in alpha ... and alpha-l-fucosidases, the enzymes responsible for their processing, are therefore of crucial importance. Deficiency in alpha ...
The FUCA1 gene provides instructions for making an enzyme called alpha-L-fucosidase. Learn about this gene and related health ... The FUCA1 gene provides instructions for making an enzyme called alpha-L-fucosidase. This enzyme is found in lysosomes, which ... Most of these mutations result in an abnormally short, nonfunctional alpha-L-fucosidase enzyme. Without this enzyme, ... Alpha-L-fucosidase is responsible for cutting (cleaving) off a sugar molecule called fucose toward the end of the breakdown ...
Leukocyte extravasation: An immunoregulatory role for alpha-L-Fucosidase?. Ali, S., Jenkins, Y., Kirkley, M., Dagkalis, A., ... Leukocyte extravasation: An immunoregulatory role for α-L-fucosidase?. Ali, S., Jenkins, Y., Kirkley, M., Dagkalis, A., ... Lewy body variant of Alzheimers disease: selective neocortical loss of t-SNARE proteins and loss of MAP2 and alpha-synuclein ...
Alpha-Fucosidase Deficiency Deficiency Disease, alpha-Fucosidase Deficiency Disease, alpha-L-Fucosidase Fucosidase Deficiency ... alpha-Fucosidase Deficiency Disease alpha-L-Fucosidase Deficiency alpha-L-Fucosidase Deficiency Disease Previous Indexing. ... Fucosidase (1975-1984). See Also. alpha-L-Fucosidase. Public MeSH Note. 1985. History Note. 1985. Date Established. 1985/01/01 ... alpha-L-Fucosidase Deficiency Disease Term UI T368134. Date11/03/1999. LexicalTag NON. ThesaurusID NLM (2000). ...
b.71.1.3: Putative alpha-L-fucosidase C-terminal domain [101928] (1 protein). glycosyl hydrolase family 29; beta-sheet forms a ... b.71.1.1: alpha-Amylases, C-terminal beta-sheet domain [51012] (21 proteins). this domain follows the catalytic beta/alpha ... interrupted by the catalytic domain; the C-terminal core is similar to the alpha-amylase domain. ... this domain is C-terminal to the catalytic beta/alpha barrel domain. ...
The Enzyme Collection contains over 550 mAbs that recognize catalytic domains or associated regulatory subunits in enyme complexes
alpha-L-fucosidase. 113. SEQF2753,CP012074.1. AKU68424.1 jb [NA] [AA] 1014/337. 172702-171689. ABC transporter ATP-binding ... electron transfer flavoprotein subunit alpha. 128. SEQF2753,CP012074.1. AKU68439.1 jb [NA] [AA] 870/289. 201198-200329. ...
Structural and thermodynamic analyses of alpha-L-fucosidase inhibitors. Login. New ideas about the solubility of drugs ...
AXY8 encodes an alpha-fucosidase, underscoring the importance of apoplastic metabolism on the fine structure of Arabidopsis ...
alpha 1-2.4.6 Fucosidase O. (New England Biolabs). 400 units Zaloguj się Dodaj do koszyka ...
Alpha-Fucosidase Deficiency Deficiency Disease, alpha-Fucosidase Deficiency Disease, alpha-L-Fucosidase Fucosidase Deficiency ... alpha-Fucosidase Deficiency Disease alpha-L-Fucosidase Deficiency alpha-L-Fucosidase Deficiency Disease Previous Indexing. ... Fucosidase (1975-1984). See Also. alpha-L-Fucosidase. Public MeSH Note. 1985. History Note. 1985. Date Established. 1985/01/01 ... alpha-L-Fucosidase Deficiency Disease Term UI T368134. Date11/03/1999. LexicalTag NON. ThesaurusID NLM (2000). ...
As the working co-authored alpha-L-fucosidase in the United States translating on number of and confirm those new site models, ...
alpha-L-fucosidase 1 [Source:HGNC Symbol.... GEM. 2669. GEM. GTP binding protein overexpressed in ske.... ...
FUCOSIDASE. ALPHA-L-FUCOSIDASE. GALACTOSEMIA. GALACTOSEMIAS. GANGLIOSIDOSIS G(M1). GANGLIOSIDOSIS GM1. GAUCHERS DISEASE. ...
alpha-Fucosidase Deficiency Diseases alpha-L-Fucosidase Deficiency alpha-L-Fucosidase Deficiency Disease alpha-L-Fucosidase ... alpha-Fucosidase Deficiency Diseases. alpha-L-Fucosidase Deficiency. alpha-L-Fucosidase Deficiency Disease. alpha-L-Fucosidase ... Fucosidase Deficiency Diseases alpha Fucosidase Deficiency Disease alpha L Fucosidase Deficiency Disease alpha-Fucosidase ... alpha L Fucosidase Deficiency Disease, alpha-Fucosidase Deficiency Disease, alpha-L-Fucosidase Deficiency Diseases, Fucosidase ...
FUCOSIDASE. ALPHA-L-FUCOSIDASE. GALACTOSEMIA. GALACTOSEMIAS. GANGLIOSIDOSIS G(M1). GANGLIOSIDOSIS GM1. GAUCHERS DISEASE. ...
Alpha-fucosidase measurement (procedure). Code System Preferred Concept Name. Alpha-fucosidase measurement (procedure). ...
FUCOSIDASE. ALPHA-L-FUCOSIDASE. GALACTOSEMIA. GALACTOSEMIAS. GANGLIOSIDOSIS G(M1). GANGLIOSIDOSIS GM1. GAUCHERS DISEASE. ...
FUCOSIDASE. ALPHA-L-FUCOSIDASE. GALACTOSEMIA. GALACTOSEMIAS. GANGLIOSIDOSIS G(M1). GANGLIOSIDOSIS GM1. GAUCHERS DISEASE. ...
FUCOSIDASE. ALPHA-L-FUCOSIDASE. GALACTOSEMIA. GALACTOSEMIAS. GANGLIOSIDOSIS G(M1). GANGLIOSIDOSIS GM1. GAUCHERS DISEASE. ...
FUCOSIDASE. ALPHA-L-FUCOSIDASE. GALACTOSEMIA. GALACTOSEMIAS. GANGLIOSIDOSIS G(M1). GANGLIOSIDOSIS GM1. GAUCHERS DISEASE. ...
FUCOSIDASE. ALPHA-L-FUCOSIDASE. GALACTOSEMIA. GALACTOSEMIAS. GANGLIOSIDOSIS G(M1). GANGLIOSIDOSIS GM1. GAUCHERS DISEASE. ...
FUCOSIDASE. ALPHA-L-FUCOSIDASE. GALACTOSEMIA. GALACTOSEMIAS. GANGLIOSIDOSIS G(M1). GANGLIOSIDOSIS GM1. GAUCHERS DISEASE. ...
FUCOSIDASE. ALPHA-L-FUCOSIDASE. GALACTOSEMIA. GALACTOSEMIAS. GANGLIOSIDOSIS G(M1). GANGLIOSIDOSIS GM1. GAUCHERS DISEASE. ...
FUCOSIDASE. ALPHA-L-FUCOSIDASE. GALACTOSEMIA. GALACTOSEMIAS. GANGLIOSIDOSIS G(M1). GANGLIOSIDOSIS GM1. GAUCHERS DISEASE. ...
FUCOSIDASE. ALPHA-L-FUCOSIDASE. GALACTOSEMIA. GALACTOSEMIAS. GANGLIOSIDOSIS G(M1). GANGLIOSIDOSIS GM1. GAUCHERS DISEASE. ...
FUCOSIDASE. ALPHA-L-FUCOSIDASE. GALACTOSEMIA. GALACTOSEMIAS. GANGLIOSIDOSIS G(M1). GANGLIOSIDOSIS GM1. GAUCHERS DISEASE. ...
FUCOSIDASE. ALPHA-L-FUCOSIDASE. GALACTOSEMIA. GALACTOSEMIAS. GANGLIOSIDOSIS G(M1). GANGLIOSIDOSIS GM1. GAUCHERS DISEASE. ...
FUCOSIDASE. ALPHA-L-FUCOSIDASE. GALACTOSEMIA. GALACTOSEMIAS. GANGLIOSIDOSIS G(M1). GANGLIOSIDOSIS GM1. GAUCHERS DISEASE. ...
  • The FUCA1 gene provides instructions for making an enzyme called alpha-L-fucosidase. (medlineplus.gov)
  • This enzyme assembles as a hexamer and displays a two-domain fold, composed of a catalytic (beta/alpha)(8)-like domain and a C-terminal beta-sandwich domain. (rcsb.org)
  • Because T. maritima alpha-l-fucosidase occupies a unique evolutionary position, being far more closely related to the mammalian enzymes than to any other prokaryotic homolog, a structural model of the human enzyme was built to document the structural consequences of the genetic mutations associated with fucosidosis. (rcsb.org)
  • Most of these mutations result in an abnormally short, nonfunctional alpha-L-fucosidase enzyme. (medlineplus.gov)
  • The enzyme transfers the amino group from glutamate to oxaloacetate producing alpha-ketoglutarate and aspartate. (smpdb.ca)
  • Dietary restriction has shown promise for disorders such as lysosomal acid lipase deficiency (Wolman disease), as has incorporation of lipid-lowering drugs in the regimen along with sebelipase alpha, a recombinant enzyme replacement therapy. (medscape.com)
  • To gain insight into alpha-l-fucosidase function at the molecular level, we have determined the crystal structure of Thermotoga maritima alpha-l-fucosidase. (rcsb.org)
  • Activity of N-acetyl-beta-D hexosoaminidase and alpha-fucosidase in differentiation of acute alcoholic pancreatitis. (edu.pl)
  • Fucosylated glycoconjugates are involved in numerous biological events, and alpha-l-fucosidases, the enzymes responsible for their processing, are therefore of crucial importance. (rcsb.org)
  • Alpha-L-fucosidase is responsible for cutting (cleaving) off a sugar molecule called fucose toward the end of the breakdown process. (medlineplus.gov)
  • Since the asparagine side chain can make efficient hydrogen bond interactions with the peptide backbone, asparagines are often found near the beginning and end of alpha-helices, and in turn motifs in beta sheets. (smpdb.ca)
  • AXY8 encodes an alpha-fucosidase, underscoring the importance of apoplastic metabolism on the fine structure of Arabidopsis cell wall polysaccharides. (mpg.de)
  • The rare lysosomal storage disease, fucosidosis results from an almost complete deficiency of alpha-L-fucosidase (EC 3.2.1.51). (uantwerpen.be)
  • This gene encodes a plasma alpha-L-fucosidase, which represents 10-20% of the total cellular fucosidase activity. (nih.gov)
  • C34816 Congenital Metabolic Disorder C99147 Neonatal Research Network Terminology C98699 5 Alpha Steroid Reductase 2 Deficiency 5 Alpha Steroid Reductase 2 Deficiency 3-Oxo-5 Alpha-Steroid Delta 4-Dehydrogenase Deficiency An autosomal recessive inherited disorder caused by mutations in the SRD5A2 gene. (nih.gov)
  • We compared AFP with serum levels of vascular endothelial growth factors (VEGF and VEGF-A), insulin- like growth factor-2 (IGF-II), and the activity of the lysosomal enzyme alpha-L-fucosidase (AFU) in the sensitivity of detection of HCC and cirrhosis in Egyptian patients. (medscape.com)
  • Among these factors is a-L-fucosidase (AFU), a lysosomal enzyme present in all mammalian cells. (medscape.com)
  • Dietary restriction has shown promise for disorders such as lysosomal acid lipase deficiency (Wolman disease), as has incorporation of lipid-lowering drugs in the regimen along with sebelipase alpha, a recombinant enzyme replacement therapy. (medscape.com)
  • An X-linked inherited metabolic disease caused by a deficiency of lysosomal ALPHA-GALACTOSIDASE A. It is characterized by intralysosomal accumulation of globotriaosylceramide and other GLYCOSPHINGOLIPIDS in blood vessels throughout the body leading to multi-system complications including renal, cardiac, cerebrovascular, and skin disorders. (lookformedical.com)
  • It is characterized by deficiency of the enzyme steroid 5-alpha reductase 2 that catalyzes the conversion of testosterone to dihydrotestosterone. (nih.gov)
  • Alpha-L-fucosidase is responsible for cutting (cleaving) off a sugar molecule called fucose toward the end of the breakdown process. (medlineplus.gov)
  • An enzyme that catalyzes the hydrolysis of an alpha L-fucoside to yield an alcohol and L-fucose. (nih.gov)
  • Alpha-L-fucosidase is responsible for hydrolyzing the alpha-1,6-linked fucose joined to the reducing-end N-acetylglucosamine of the carbohydrate moieties of glycoproteins. (nih.gov)
  • The protein is a member of the glycosyl hydrolase 29 family, and catalyzes the hydrolysis of the alpha-1,6-linked fucose joined to the reducing-end N-acetylglucosamine of the carbohydrate moieties of glycoproteins. (nih.gov)
  • We show that the F-type lectin domain of this protein recognizes fucosylated glycans in both α and β linkages but has high affinity for a Fuc-α-1,2-Gal motif and that the alpha-l-fucosidase domain displays hydrolytic activity on glycan substrates with α1-2 and α1-4 linked fucose. (harvard.edu)
  • We also show that the F-type lectin domain does not have any effect on the activity of the cis-positioned alpha-l-fucosidase domain with the synthetic substrate, 4-Methylumbelliferyl-alpha-l-fucopyranoside or on inhibition of this activity by l-fucose or deoxyfuconojirimycin hydrochloride. (harvard.edu)
  • 6. Serum midkine is a more sensitive predictor for hepatocellular carcinoma than Dickkopf-1 and alpha-L-fucosidase in cirrhotic HCV patients. (nih.gov)
  • 7. Alpha-L-fucosidase as a serum marker of hepatocellular carcinoma in Thailand. (nih.gov)
  • 13. A comparison of glypican-3 with alpha-fetoprotein as a serum marker for hepatocellular carcinoma: a meta-analysis. (nih.gov)
  • 16. [A preliminary study on serum alpha-L-fucosidase assay in the diagnosis of hepatocellular carcinoma]. (nih.gov)
  • 18. [Value of the serum measurement of alpha-L-fucosidase in the diagnosis of hepatocarcinoma]. (nih.gov)
  • 19. Serum alpha-L-fucosidase. (nih.gov)
  • 20. [Diagnostic significance of the determination of serum alpha-L-fucosidase in primary hepatocellular carcinoma]. (nih.gov)
  • Liu A , Li Y , Shen L , Shen L , Li Z , . Clinical utility of serum fucosylated fraction of alpha-fetoprotein in the diagnostic of hepatocellular carcinoma: a comprehensive analysis with large sample size. (aging-us.com)
  • Serum alpha fetoprotein (AFP) is the most widely used tumor marker in detecting patients with hepatocellular carcinoma, and has been proven to have capability of prefiguring the prognosis. (nih.gov)
  • 1. Diagnostic value of alpha-L-fucosidase for hepatocellular carcinoma: a meta-analysis. (nih.gov)
  • 2. Evaluation of squamous cell carcinoma antigen-immunoglobulin M complex (SCCA-IGM) and alpha-L-fucosidase (AFU) as novel diagnostic biomarkers for hepatocellular carcinoma. (nih.gov)
  • 4. Clinical performance of α-L-fucosidase for early detection of hepatocellular carcinoma. (nih.gov)
  • 5. Alpha-fetoprotein-L3 in hepatocellular carcinoma: a meta-analysis. (nih.gov)
  • 9. Alpha-L-fucosidase as a tumour marker of hepatocellular carcinoma. (nih.gov)
  • 10. Golgi protein 73 versus alpha-fetoprotein as a biomarker for hepatocellular carcinoma: a diagnostic meta-analysis. (nih.gov)
  • 12. Evaluation of the diagnostic value of alpha-l-fucosidase, alpha-fetoprotein and thymidine kinase 1 with ROC and logistic regression for hepatocellular carcinoma. (nih.gov)
  • 14. Alpha-1-fucosidase as a prognostic indicator for hepatocellular carcinoma following hepatectomy: a large-scale, long-term study. (nih.gov)
  • While alpha-fetoprotein (AFP) is a commonly used tumor marker in the detection of hepatocellular carcinoma (HCC), its sensitivity and specificity are insufficient to detect HCC in all patient samples. (medscape.com)
  • ORF of fucosidase, alpha-L- 2, plasma (FUCA2) in pENTER vector with CMV promoter and C-terminal FLAG and His tags. (criver.com)
  • Dog GJA1/Gap junction alpha-1 protein ELISA Kitis available at Gentaur for Next week Delivery. (researchd.com)
  • Specificity: Natural and recombinant Dog Gap junction alpha-1 protein GeneName: GJA1 ProteinName: Gap junction alpha-1 protein Alternative: Connexin-43,Cx43. (researchd.com)
  • Here, we report the biochemical characterization of a Streptosporangium roseum protein containing an F-type lectin domain in tandem with an NPCBM-associated domain and a family GH 29A alpha-l-fucosidase domain. (harvard.edu)
  • In previous studies we described a decreased alpha-L-fucosidase activity in colorectal tumors, appearing as a prognostic factor of tumoral recurrence. (nih.gov)
  • infantis and Bifidobacterium bifidum, possess an arsenal of enzymes including α-fucosidases, sialidases, and β-galactosidases to metabolise HMOs. (biomedcentral.com)
  • A group of enzymes that catalyze the hydrolysis of alpha- or beta-xylosidic linkages. (wakehealth.edu)
  • Among the various methods of HCC screening, assays based on the detection of biomarker that is specific to HCC such as alpha-L-fucosidase (AFU) have been regarded as the most prominent methods. (elsevier.com)
  • Furthermore, some other tumor markers, such as glypican-3, gamma-glutamyl transferase II, alpha-l-fucosidase, transforming growth factor-beta1, tumor-specific growth factor, have been indicated to be available supplementaries to AFP in the detection. (nih.gov)
  • Alpha amylase is classified as family 13 of the glycosyl hydrolases. (xfam.org)
  • Other xylosidases have been identified that catalyze the hydrolysis of alpha-xylosidic bonds. (wakehealth.edu)
  • Molecular basis of the common electrophoretic polymorphism (Fu1/Fu2) in human alpha-L-fucosidase. (nih.gov)
  • infantis TPY11-2 harboured four α-fucosidases with 95-99 % similarity to the type strain. (biomedcentral.com)
  • The structure is an 8 stranded alpha/beta barrel containing the active site, interrupted by a ~70 a.a. calcium-binding domain protruding between beta strand 3 and alpha helix 3, and a carboxyl-terminal Greek key beta-barrel domain. (xfam.org)
  • Dog FGA/Fibrinogen alpha chain ELISA Kitis available at Gentaur for Next week Delivery. (researchd.com)