alpha-L-Fucosidase
Fucosidosis
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)
Lymphocyte Function-Associated Antigen-1
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.
Antigens, CD18
Integrins
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
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.
Molecular Sequence Data
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.
Amino Acid Sequence
Base Sequence
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)
1 3 alpha l fucosidase : définition de 1 3 alpha l fucosidase et synonymes de 1 3 alpha l fucosidase (anglais)
An anthracenecarboximide fluorescent probe for in vitro and in vivo ratiometric imaging of endogenous alpha-l-fucosidase for...
Identifying the Catalytic Acid/Base in GH29 alpha-L-Fucosidase Subfamilies | UBC Chemistry
AID 36841 - Inhibition of the enzyme alpha-L-fucosidase from bovine epididymis was determined at 1 mM concentration of the...
KEGG BRITE: KEGG Orthology (KO) - Streptococcus pneumoniae TIGR4 (virulent serotype 4)
Synthesis and evaluation of a 5-Membered Isoiminosugar as Glycosidase Inhibitor<...
Cell surface associated alpha-L-fucose moieties modulate human breast cancer neoplastic progression
Canser yr afu - Wicipedia
FUCA1P1 Gene - GeneCards | FUCA1P1 Pseudogene
Fucosidases
A Comparative Study of Inductive Influence on the Hydrolysis of Beta-T by Robert W. Miller Jr.
FUCA1 Gene - GeneCards | FUCO Protein | FUCO Antibody
Isoenzymes of human liver α-L-fucosidase: Chemical relationship, kinetic studies, and immunochemical characterization |...
Bacillus sp. ES-SL-2 | Semantic Scholar
CAZy - GH30 1
CAZy - GH30 3
FUCA2 knockout cell line
2IWV | Genus
3RBH | Genus
Fucosidosis: genetic and biochemical analysis of eight cases. | Journal of Medical Genetics
Adelaide Research & Scholarship: Canine fucosidosis: a model for retroviral gene transfer into haematopoietic stem cells
Structural and enzymatic characterization of a glycoside hydrolase family 31 alpha-xylosidase from <em>Cellvibrio japonicus</em...
A mouse model for fucosidosis recapitulates storage pathology and neurological features of the milder form of the human disease...
Treatment of canine fucosidosis by intracisternal enzyme infusion - Fingerprint - Research @ Flinders
Synthesis and glycosidase inhibitory activities of 2-(aminoalkyl)pyrrolidine-3,4-diol derivatives
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Sperm-associated antigen elisa and antibody
Cathepsin D-like catalytic domain (IPR034129) | InterPro | EMBL-EBI
Sperm-associated antigen 11 variant E - Creative Peptides
Recombinant Human FUCA1 protein (ab207120) | Abcam
Tissue expression of FUCA2 - Staining in caudate - The Human Protein Atlas
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Sperm-associated acrosin inhibitor elisa and antibody
The Turkish Journal of Pediatrics
Salivary Exoglycosidases as Markers of Alcohol Dependence | New Hope Foundation
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Tissue expression of FUCA2 - Staining in endometrium - The Human Protein Atlas
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Alpha-fucosidase
... may refer to one of two enzymes: Fucosidase Alpha-L-fucosidase This set index page lists enzyme articles ...
Alpha-L-fucosidase
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 ...
1,2-alpha-L-fucosidase
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 ...
1,3-alpha-L-fucosidase
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 ...
1,6-alpha-L-fucosidase
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 ...
Fucosidase
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 ...
Glycoside hydrolase family 29
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 ...
FUCA2
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 ...
Fucosidosis
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 ...
Borrelia burgdorferi
"Alpha fucosidase and beta galactosidase in serum of a Lyme disease patients as a possible marker of accelerated senescence - a ...
Thymidine kinase in clinical chemistry
Zhang SY, Lin BD, Li BR (2015). "Evaluation of the diagnostic value of alpha-l-fucosidase, alpha-fetoprotein and thymidine ...
Penicillium multicolor
"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 ...
Fucoidanase
... 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 ...
List of MeSH codes (D08)
... 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. ...
Pseudogymnoascus destructans
... alpha/beta-galactosidase, β-glucuronidase, α-fucosidase, α-mannosidase, and trypsin. Important dual virulence factors found in ...
Beta-D-fucosidase
... 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 ...
RCSB PDB - 1HL9: CRYSTAL STRUCTURE OF THERMOTOGA MARITIMA ALPHA-FUCOSIDASE IN COMPLEX WITH A MECHANISM BASED INHIBITOR
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 ...
FUCA1 gene: MedlinePlus Genetics
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 ...
Find Research outputs
- The University of Aberdeen Research Portal
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 ...
MeSH Browser
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). ...
SCOP 1.71: Superfamily b.71.1: Glycosyl hydrolase domain
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. ...
Isotype: RIgG2a, Clonality: Monoclonal
HOMD :: SEQF2753
Biorelevant Dissolution | Pion
Publications of Yariv Brotman | Max Planck Institute of Molecular Plant Physiology
MeSH Browser
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). ...
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DeCS - Changed terms
DeCS
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 ...
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Code System Concept
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FUCA11
- The FUCA1 gene provides instructions for making an enzyme called alpha-L-fucosidase. (medlineplus.gov)
Enzyme5
- 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)
Thermotoga1
- 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)
Activity1
- Activity of N-acetyl-beta-D hexosoaminidase and alpha-fucosidase in differentiation of acute alcoholic pancreatitis. (edu.pl)
Responsible2
- 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)
Beta1
- 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)
Importance1
- AXY8 encodes an alpha-fucosidase, underscoring the importance of apoplastic metabolism on the fine structure of Arabidopsis cell wall polysaccharides. (mpg.de)
1.511
- The rare lysosomal storage disease, fucosidosis results from an almost complete deficiency of alpha-L-fucosidase (EC 3.2.1.51). (uantwerpen.be)
Gene2
- 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)
Lysosomal4
- 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)
Deficiency1
- It is characterized by deficiency of the enzyme steroid 5-alpha reductase 2 that catalyzes the conversion of testosterone to dihydrotestosterone. (nih.gov)
Fucose6
- 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)
Serum9
- 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)
Hepatocellular9
- 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)
Fuca21
- ORF of fucosidase, alpha-L- 2, plasma (FUCA2) in pENTER vector with CMV promoter and C-terminal FLAG and His tags. (criver.com)
Protein3
- 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)
Prognostic1
- In previous studies we described a decreased alpha-L-fucosidase activity in colorectal tumors, appearing as a prognostic factor of tumoral recurrence. (nih.gov)
Enzymes2
- 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)
Detection2
- 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)
Hydrolases1
- Alpha amylase is classified as family 13 of the glycosyl hydrolases. (xfam.org)
Hydrolysis1
- Other xylosidases have been identified that catalyze the hydrolysis of alpha-xylosidic bonds. (wakehealth.edu)
Human1
- Molecular basis of the common electrophoretic polymorphism (Fu1/Fu2) in human alpha-L-fucosidase. (nih.gov)
Type1
- infantis TPY11-2 harboured four α-fucosidases with 95-99 % similarity to the type strain. (biomedcentral.com)
Domain1
- 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)
Chain1
- Dog FGA/Fibrinogen alpha chain ELISA Kitis available at Gentaur for Next week Delivery. (researchd.com)