Complement C3c: A 206-amino-acid fragment in the alpha chain (672-1663) of C3b. It is generated when C3b is inactivated (iC3b) and its alpha chain is cleaved by COMPLEMENT FACTOR I into C3c (749-954), and C3dg (955-1303) in the presence COMPLEMENT FACTOR H.alpha-Galactosidase: An enzyme that catalyzes the hydrolysis of terminal, non-reducing alpha-D-galactose residues in alpha-galactosides including galactose oligosaccharides, galactomannans, and galactolipids.Fabry Disease: 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.Galactosidases: A family of galactoside hydrolases that hydrolyze compounds with an O-galactosyl linkage. EC 3.2.1.-.beta-Galactosidase: A group of enzymes that catalyzes the hydrolysis of terminal, non-reducing beta-D-galactose residues in beta-galactosides. Deficiency of beta-Galactosidase A1 may cause GANGLIOSIDOSIS, GM1.Cerebrosides: Neutral glycosphingolipids that contain a monosaccharide, normally glucose or galactose, in 1-ortho-beta-glycosidic linkage with the primary alcohol of an N-acyl sphingoid (ceramide). In plants the monosaccharide is normally glucose and the sphingoid usually phytosphingosine. In animals, the monosaccharide is usually galactose, though this may vary with the tissue and the sphingoid is usually sphingosine or dihydrosphingosine. (From Oxford Dictionary of Biochemistry and Molecular Biology, 1st ed)Lactose: A disaccharide of GLUCOSE and GALACTOSE in human and cow milk. It is used in pharmacy for tablets, in medicine as a nutrient, and in industry.Galactose: An aldohexose that occurs naturally in the D-form in lactose, cerebrosides, gangliosides, and mucoproteins. Deficiency of galactosyl-1-phosphate uridyltransferase (GALACTOSE-1-PHOSPHATE URIDYL-TRANSFERASE DEFICIENCY DISEASE) causes an error in galactose metabolism called GALACTOSEMIA, resulting in elevations of galactose in the blood.Acridine Orange: A cationic cytochemical stain specific for cell nuclei, especially DNA. It is used as a supravital stain and in fluorescence cytochemistry. It may cause mutations in microorganisms.alpha-L-Fucosidase: 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.Nigericin: A polyether antibiotic which affects ion transport and ATPase activity in mitochondria. It is produced by Streptomyces hygroscopicus. (From Merck Index, 11th ed)Glycoside HydrolasesGlycosides: Any compound that contains a constituent sugar, in which the hydroxyl group attached to the first carbon is substituted by an alcoholic, phenolic, or other group. They are named specifically for the sugar contained, such as glucoside (glucose), pentoside (pentose), fructoside (fructose), etc. Upon hydrolysis, a sugar and nonsugar component (aglycone) are formed. (From Dorland, 28th ed; From Miall's Dictionary of Chemistry, 5th ed)Cell Aging: The decrease in the cell's ability to proliferate with the passing of time. Each cell is programmed for a certain number of cell divisions and at the end of that time proliferation halts. The cell enters a quiescent state after which it experiences CELL DEATH via the process of APOPTOSIS.Histocytochemistry: Study of intracellular distribution of chemicals, reaction sites, enzymes, etc., by means of staining reactions, radioactive isotope uptake, selective metal distribution in electron microscopy, or other methods.Escherichia coli: A species of gram-negative, facultatively anaerobic, rod-shaped bacteria (GRAM-NEGATIVE FACULTATIVELY ANAEROBIC RODS) commonly found in the lower part of the intestine of warm-blooded animals. It is usually nonpathogenic, but some strains are known to produce DIARRHEA and pyogenic infections. Pathogenic strains (virotypes) are classified by their specific pathogenic mechanisms such as toxins (ENTEROTOXIGENIC ESCHERICHIA COLI), etc.alpha 1-Antitrypsin: Plasma glycoprotein member of the serpin superfamily which inhibits TRYPSIN; NEUTROPHIL ELASTASE; and other PROTEOLYTIC ENZYMES.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.Receptors, Adrenergic, alpha: One of the two major pharmacological subdivisions of adrenergic receptors that were originally defined by the relative potencies of various adrenergic compounds. The alpha receptors were initially described as excitatory receptors that post-junctionally stimulate SMOOTH MUSCLE contraction. However, further analysis has revealed a more complex picture involving several alpha receptor subtypes and their involvement in feedback regulation.Hypoxia-Inducible Factor 1, alpha Subunit: Hypoxia-inducible factor 1, alpha subunit is a basic helix-loop-helix transcription factor that is regulated by OXYGEN availability and is targeted for degradation by VHL TUMOR SUPPRESSOR PROTEIN.alpha7 Nicotinic Acetylcholine Receptor: A member of the NICOTINIC ACETYLCHOLINE RECEPTOR subfamily of the LIGAND-GATED ION CHANNEL family. It consists entirely of pentameric a7 subunits expressed in the CNS, autonomic nervous system, vascular system, lymphocytes and spleen.Integrin alpha3beta1: Cell surface receptor for LAMININ, epiligrin, FIBRONECTINS, entactin, and COLLAGEN. Integrin alpha3beta1 is the major integrin present in EPITHELIAL CELLS, where it plays a role in the assembly of BASEMENT MEMBRANE as well as in cell migration, and may regulate the functions of other integrins. Two alternatively spliced isoforms of the alpha subunit (INTEGRIN ALPHA3), are differentially expressed in different cell types.Integrin alpha4: An integrin alpha subunit that is unique in that it does not contain an I domain, and its proteolytic cleavage site is near the middle of the extracellular portion of the polypeptide rather than close to the membrane as in other integrin alpha subunits.Integrin alpha6: An integrin alpha subunit that primarily associates with INTEGRIN BETA1 or INTEGRIN BETA4 to form laminin-binding heterodimers. Integrin alpha6 has two alternatively spliced isoforms: integrin alpha6A and integrin alpha6B, which differ in their cytoplasmic domains and are regulated in a tissue-specific and developmental stage-specific manner.Integrin alpha5beta1: An integrin found in FIBROBLASTS; PLATELETS; MONOCYTES, and LYMPHOCYTES. Integrin alpha5beta1 is the classical receptor for FIBRONECTIN, but it also functions as a receptor for LAMININ and several other EXTRACELLULAR MATRIX PROTEINS.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.Interleukin-1alpha: An interleukin-1 subtype that occurs as a membrane-bound pro-protein form that is cleaved by proteases to form a secreted mature form. Unlike INTERLEUKIN-1BETA both membrane-bound and secreted forms of interleukin-1alpha are biologically active.Integrin alpha2beta1: An integrin found on fibroblasts, platelets, endothelial and epithelial cells, and lymphocytes where it functions as a receptor for COLLAGEN and LAMININ. Although originally referred to as the collagen receptor, it is one of several receptors for collagen. Ligand binding to integrin alpha2beta1 triggers a cascade of intracellular signaling, including activation of p38 MAP kinase.Receptors, Adrenergic, alpha-1: A subclass of alpha-adrenergic receptors that mediate contraction of SMOOTH MUSCLE in a variety of tissues such as ARTERIOLES; VEINS; and the UTERUS. They are usually found on postsynaptic membranes and signal through GQ-G11 G-PROTEINS.Diphtheria-Tetanus-Pertussis Vaccine: A vaccine consisting of DIPHTHERIA TOXOID; TETANUS TOXOID; and whole-cell PERTUSSIS VACCINE. The vaccine protects against diphtheria, tetanus, and whooping cough.Rotavirus Vaccines: Vaccines or candidate vaccines used to prevent infection with ROTAVIRUS.Immunization Schedule: Schedule giving optimum times usually for primary and/or secondary immunization.Vaccines, Combined: Two or more vaccines in a single dosage form.Diphtheria-Tetanus-acellular Pertussis Vaccines: Combined vaccines consisting of DIPHTHERIA TOXOID; TETANUS TOXOID; and an acellular form of PERTUSSIS VACCINE. At least five different purified antigens of B. pertussis have been used in various combinations in these vaccines.PubMed: A bibliographic database that includes MEDLINE as its primary subset. It is produced by the National Center for Biotechnology Information (NCBI), part of the NATIONAL LIBRARY OF MEDICINE. PubMed, which is searchable through NLM's Web site, also includes access to additional citations to selected life sciences journals not in MEDLINE, and links to other resources such as the full-text of articles at participating publishers' Web sites, NCBI's molecular biology databases, and PubMed Central.Periodicals as Topic: A publication issued at stated, more or less regular, intervals.BooksPublishing: "The business or profession of the commercial production and issuance of literature" (Webster's 3d). It includes the publisher, publication processes, editing and editors. Production may be by conventional printing methods or by electronic publishing.MEDLINE: The premier bibliographic database of the NATIONAL LIBRARY OF MEDICINE. MEDLINE® (MEDLARS Online) is the primary subset of PUBMED and can be searched on NLM's Web site in PubMed or the NLM Gateway. MEDLINE references are indexed with MEDICAL SUBJECT HEADINGS (MeSH).Databases, Protein: Databases containing information about PROTEINS such as AMINO ACID SEQUENCE; PROTEIN CONFORMATION; and other properties.Internet: A loose confederation of computer communication networks around the world. The networks that make up the Internet are connected through several backbone networks. The Internet grew out of the US Government ARPAnet project and was designed to facilitate information exchange.User-Computer Interface: The portion of an interactive computer program that issues messages to and receives commands from a user.Crystallography, X-Ray: The study of crystal structure using X-RAY DIFFRACTION techniques. (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)Models, Molecular: Models used experimentally or theoretically to study molecular shape, electronic properties, or interactions; includes analogous molecules, computer-generated graphics, and mechanical structures.Software: Sequential operating programs and data which instruct the functioning of a digital computer.Antibodies: Immunoglobulin molecules having a specific amino acid sequence by virtue of which they interact only with the ANTIGEN (or a very similar shape) that induced their synthesis in cells of the lymphoid series (especially PLASMA CELLS).Angiokeratoma: A vascular, horny neoplasm of the skin characterized by TELANGIECTASIS and secondary epithelial changes including acanthosis and hyperkeratosis.Pathology, Molecular: A subspecialty of pathology concerned with the molecular basis (e.g., mutations) of various diseases.Pseudotsuga: A plant genus in the family PINACEAE, order Pinales, class Pinopsida, division Coniferophyta. They are coniferous evergreen trees with long, flat, spirally arranged needles that grow directly from the branch.Trihexosylceramides: Glycosphingolipids which contain as their polar head group a trisaccharide (galactose-galactose-glucose) moiety bound in glycosidic linkage to the hydroxyl group of ceramide. Their accumulation in tissue, due to a defect in ceramide trihexosidase, is the cause of angiokeratoma corporis diffusum (FABRY DISEASE).Eye Manifestations: Ocular disorders attendant upon non-ocular disease or injury.

Lignocellulose degradation by Phanerochaete chrysosporium: purification and characterization of the main alpha-galactosidase. (1/449)

The main alpha-galactosidase was purified to homogeneity, in 30% yield, from a solid culture of Phanerochaete chrysosporium on 1 part wheat bran/2 parts thermomechanical softwood pulp. It is a glycosylated tetramer of 50 kDa peptide chains, which gives the N-terminal sequence ADNGLAITPQMG(?W)NT(?W)NHFG(?W)DIS(?W)DTI. It is remarkably stable, with crude extracts losing no activity over 3 h at 80 degrees C, and the purified enzyme retaining its activity over several months at 4 degrees C. The kinetics of hydrolysis at 25 degrees C of various substrates by this retaining enzyme were measured, absolute parameters being obtained by active-site titration with 2',4',6'-trinitrophenyl 2-deoxy-2, 2-difluoro-alpha-D-galactopyranoside. The variation of kcat/Km for 1-naphthyl-alpha-D-galactopyranoside with pH is bell-shaped, with pK1=1.91 and pK2=5.54. The alphaD(V/K) value for p-nitrophenyl-alpha-D-glucopyranoside is 1.031+/-0.007 at the optimal pH of 3.75 and 1.114+/-0.006 at pH7.00, indicating masking of the intrinsic effect at optimal pH. There is no alpha-2H effect on binding galactose [alphaD(Ki)=0.994+/-0.013]. The enzyme hydrolyses p-nitrophenyl beta-L-arabinopyranoside approximately 510 times slower than the galactoside, but has no detectable activity on the alpha-D-glucopyranoside or alpha-D-mannopyranoside. Hydrolysis of alpha-galactosides with poor leaving groups is Michaelian, but that of substrates with good leaving groups exhibits pronounced apparent substrate inhibition, with Kis values similar to Km values. We attribute this to the binding of the second substrate molecule to a beta-galactopyranosyl-enzyme intermediate, forming an E.betaGal. alphaGalX complex which turns over slowly, if at all. 1-Fluoro-alpha-D-galactopyranosyl fluoride, unlike alpha-D-galactopyranosyl fluoride, is a Michaelian substrate, indicating that the effect of 1-fluorine substitution is greater on the first than on the second step of the enzyme reaction.  (+info)

Aging accentuates and bone marrow transplantation ameliorates metabolic defects in Fabry disease mice. (2/449)

Fabry disease is an X-linked metabolic disorder caused by a deficiency of alpha-galactosidase A (alpha-Gal A). The enzyme defect leads to the systemic accumulation of glycosphingolipids with alpha-galactosyl moieties consisting predominantly of globotriaosylceramide (Gb3). In patients with this disorder, glycolipid deposition in endothelial cells leads to renal failure and cardiac and cerebrovascular disease. Recently, we generated alpha-Gal A gene knockout mouse lines and described the phenotype of 10-week-old mice. In the present study, we characterize the progression of the disease with aging and explore the effects of bone marrow transplantation (BMT) on the phenotype. Histopathological analysis of alpha-Gal A -/0 mice revealed subclinical lesions in the Kupffer cells in the liver and macrophages in the skin with no gross lesions in the endothelial cells. Gb3 accumulation and pathological lesions in the affected organs increased with age. Treatment with BMT from the wild-type mice resulted in the clearance of accumulated Gb3 in the liver, spleen, and heart with concomitant elevation of alpha-Gal A activity. These findings suggest that BMT may have a potential role in the management of patients with Fabry disease.  (+info)

Differential expression of three alpha-galactosidase genes and a single beta-galactosidase gene from Aspergillus niger. (3/449)

A gene encoding a third alpha-galactosidase (AglB) from Aspergillus niger has been cloned and sequenced. The gene consists of an open reading frame of 1,750 bp containing six introns. The gene encodes a protein of 443 amino acids which contains a eukaryotic signal sequence of 16 amino acids and seven putative N-glycosylation sites. The mature protein has a calculated molecular mass of 48,835 Da and a predicted pI of 4.6. An alignment of the AglB amino acid sequence with those of other alpha-galactosidases revealed that it belongs to a subfamily of alpha-galactosidases that also includes A. niger AglA. A. niger AglC belongs to a different subfamily that consists mainly of prokaryotic alpha-galactosidases. The expression of aglA, aglB, aglC, and lacA, the latter of which encodes an A. niger beta-galactosidase, has been studied by using a number of monomeric, oligomeric, and polymeric compounds as growth substrates. Expression of aglA is only detected on galactose and galactose-containing oligomers and polymers. The aglB gene is expressed on all of the carbon sources tested, including glucose. Elevated expression was observed on xylan, which could be assigned to regulation via XlnR, the xylanolytic transcriptional activator. Expression of aglC was only observed on glucose, fructose, and combinations of glucose with xylose and galactose. High expression of lacA was detected on arabinose, xylose, xylan, and pectin. Similar to aglB, the expression on xylose and xylan can be assigned to regulation via XlnR. All four genes have distinct expression patterns which seem to mirror the natural substrates of the encoded proteins.  (+info)

Molecular cloning of a human UDP-galactose:GlcNAcbeta1,3GalNAc beta1, 3 galactosyltransferase gene encoding an O-linked core3-elongation enzyme. (4/449)

Using the full-length amino-acid sequences of the human beta1,3 galactosyltransferase (beta3GalT)-I, -II and III enzymes as query, we have identified an additional member of the beta3GalT gene family within a sequenced region of the human chromosome 21 as found in GenBank. The novel human beta3GalT-V gene included an open reading frame of 933 bp encoding a protein of 310 amino acids with a short N-terminal cytoplasmic tail, a single predicted transmembrane domain and a large lumenal catalytic domain. The human beta3GalT-V protein showed 34%, 27%, 31% and 23% sequence identity with the human beta3GalT-I, -II, -III and -IV enzymes, respectively. The expression of beta3GalT-V as a recombinant protein in Sf9 insect cells confirmed the galactosyltransferase activity catalyzed by this enzyme. Similarly to beta3GalT-I, -II and -III, the beta3GalT-V enzyme used beta-linked GlcNAc as an acceptor, but unlike the former enzymes beta3GalT-V exhibited a marked preference for the O-linked core3 GlcNAcbeta1,3GalNAc substrate. The beta3GalT-V gene was mainly expressed in human small intestine and to a lesser extent in pancreas and testis. Although beta3GalT-V transcripts were not detected in normal colon tissue, based on Northern analysis, beta3GalT-V mRNA was found in the adenocarcinoma cell line Colo 205.  (+info)

Fifteen-year follow-up of a heterozygous Fabry's disease patient associated with pre-excitation syndrome. (5/449)

A 47-year-old woman with heterozygous Fabry's disease with pre-excitation syndrome has been followed up for 15 years. Diagnosis was confirmed by the typical electron microscopic feature of the endomyocardial specimen and a decreased plasma alpha-galactosidase activity. As the disease progressed, the interventricular septum thickened from 11 to 17 mm as measured by echocardiography, while the AH interval was prolonged from 80 to 140 msec. In Fabry's disease, the PR interval has been reported to be variable from short PR to AV block. Therefore, this case may be helpful to understand the time course in the AV conduction abnormalities with the progression of Fabry's disease.  (+info)

Purification and characterization of recombinant Mortierella vinacea alpha-galactosidases I and II expressed in Saccharomyces cerevisiae. (6/449)

The cDNAs coding for Mortierella vinacea alpha-galactosidases I and II were expressed in Saccharomyces cerevisiae under the control of the yeast GAL10 promoter. The recombinant enzymes purified to homogeneity from the culture filtrate were glycosylated, and had properties identical to those of the native enzymes except for improving the heat stability of alpha-galactosidase II and decreasing the specific activities of both enzymes.  (+info)

Cloning of the gene encoding a novel thermostable alpha-galactosidase from Thermus brockianus ITI360. (7/449)

An alpha-galactosidase gene from Thermus brockianus ITI360 was cloned, sequenced, and expressed in Escherichia coli, and the recombinant protein was purified. The gene, designated agaT, codes for a 476-residue polypeptide with a calculated molecular mass of 53, 810 Da. The native structure of the recombinant enzyme (AgaT) was estimated to be a tetramer. AgaT displays amino acid sequence similarity to the alpha-galactosidases of Thermotoga neapolitana and Thermotoga maritima and a low-level sequence similarity to alpha-galactosidases of family 36 in the classification of glycosyl hydrolases. The enzyme is thermostable, with a temperature optimum of activity at 93 degrees C with para-nitrophenyl-alpha-galactopyranoside as a substrate. Half-lives of inactivation at 92 and 80 degrees C are 100 min and 17 h, respectively. The pH optimum is between 5.5 and 6.5. The enzyme displayed high affinity for oligomeric substrates. The K(m)s for melibiose and raffinose at 80 degrees C were determined as 4.1 and 11.0 mM, respectively. The alpha-galactosidase gene in T. brockianus ITI360 was inactivated by integrational mutagenesis. Consequently, no alpha-galactosidase activity was detectable in crude extracts of the mutant strain, and it was unable to use melibiose or raffinose as a single carbohydrate source.  (+info)

Gene assignment in the spider monkey (Ateles paniscus chamek--APC): APE-MYH7 to 2q; AR-GLA-F8C to the X chromosome. (8/449)

Comparative gene assignment between the spider monkey species Ateles paniscus chamek (APC) and man (HSA) showed conserved syntenic associations despite extensive karyotypic rearrangement between species. Two HSA 14q genes were allocated to APC 2q, being syntenic to other HSA 14q and HSA 15q markers previously assigned to APC 2q, and to HSA 12q genes previously assigned to APC 2p. These findings were consistent with A. geoffroyi chromosome painting with human whole-chromosome probes, indicating that the genus Ateles is karyotypically very rearranged. On the other hand, three human X-linked markers were assigned to the Ateles X chromosome, indicating that this chromosome is evolutionary stable.  (+info)

Ninety-five of 121 (79%) adult patients and 11 of 16 (69%) pediatric patients (106 of 137, 74% of all patients) treated with Fabrazyme (agalsidase beta) in clinical studies have developed IgG antibodies to Fabrazyme (agalsidase beta) . Most patients who develop IgG antibodies do so within the first 3 months of exposure. IgG seroconversion in pediatric patients was associated with prolonged half-life of Fabrazyme (agalsidase beta) , a phenomenon rarely observed in adult patients [see CLINICAL PHARMACOLOGY and Use in Specific Populations]. A possible cause for this prolongation likely pertains to the ability of antibodies to act as "carriers" for their antigens. Among the 14 female patients exposed to Fabrazyme (agalsidase beta) in clinical studies, four (two adult and two pediatric patients) developed IgG antibodies to Fabrazyme (agalsidase beta) . IgG antibodies to Fabrazyme (agalsidase beta) were purified from 15 patients with high antibody titers ( ≥ 12,800) and studied for inhibition of in ...
Agalsidase beta is a man-made form of the naturally-occurring alpha-galactosidase A enzyme. A deficiency of this enzyme is called Fabry disease. Agalsidase beta reduces deposits of globotriaosylceramide (GL-3) in the kidneys and certain other cells in the body. Agalsidase beta is used in the treatment of Fabry disease...
Product Name:alpha-Galactosidase 4A from Bacillus halodurans CAS Number: Catalouge Number:BIZY1000 Purity: Commodity Code:99999999 MDL Number: Notes:alpha-Galactosidases are enzymes that participate in the hydrolysis of terminal, non-reducing alpha-D-galactose. Family: GH4. E.C.:3.2.1.22. Architecture: GH4. Main activity:alpha-Galactosidase. Synonyms:
Defects in the enzyme alpha-galactosidase lead to the buildup of globotriaosylceramide, causing Fabrys disease.[3] The pharmaceutical drug migalastat enhances the function of alpha-galactosidase and is used to treat Fabrys. Globotriaosylceramide is also one of the targets of Shiga toxin, which is responsible for pathogenicity of enterohemorrhagic Escherichia coli (EHEC). The bacterial Shiga toxin can be used for targeted therapy of gastric cancer, because this tumor entity expresses the receptor of the Shiga toxin. For this purpose an unspecific chemotherapeutical is conjugated to the B-subunit to make it specific. In this way only the tumor cells, but not healthy cells should be destroyed during therapy.[4] ...
Learn about Fabrazyme (Agalsidase Beta) may treat, uses, dosage, side effects, drug interactions, warnings, patient labeling, reviews, and related medications.
Semantic Scholar extracted view of Molecular pathology of Fabrys disease. Physical and kinetic properties of alpha-galactosidase A in cultured human endothelial cells. by Douglas L. Johnson et al.
Atypical hemolytic-uremic syndrome (aHUS) is a rare, potentially lethal systemic disorder, capable of affecting both adults and children, causing thrombotic microangiopathy (TMA) that leads to the formation of thrombus within small blood vessels with multiple organ failure. The pathogenesis of the aHUS is part of a sort of chronic and uncontrolled activation of the complement system by genetic mutation of some proteins usually responsible for its self-regulation. Today, the rapid diagnosis of the disease and the timely start of treatment with eculizumab, improve outcomes of renal failure, stroke and heart attack. Fabry disease is a rare tesaurismosis, X linked, due to the deficiency of the lysosomal enzyme alpha-galactosidase A, necessary for the physiological catabolism of glycosphingolipids. Multisystem clinical manifestations lead to a serious degenerative pathology. The diagnostic suspicion based on anamnesis and careful research of the symptoms and then confirmed by the enzymatic dosage of ...
AGAW : Diagnosis of Fabry disease in male patients   Verifying abnormal serum alpha-galactosidase results in male patients with a clinical presentation suggestive of Fabry disease
A natural IgG antibody (anti-Gal) with alpha-galactosyl binding specificity has been found in large amounts (0.5 - 1.0% of serum IgG) in all individuals tested. It has been purified by affinity chromatography on a column of melibiose-Sepharose. In addition to its affinity for normal and pathological senescent human red cells, the antibody readily interacts with rabbit red blood cell (RRBC) glycolipids with alpha-galactosyl terminal residues. Two types (glycosidic linkages of 1----3 vs. 1----4) of rabbit red cells glycolipids with terminal alpha-galactosyl residues were tested for antibody binding. The antibody specifically bound to glycolipids with Gal alpha 1----3 terminal residues, and treatment of these glycolipids with alpha-galactosidase abolished binding. Hemagglutination inhibition studies with oligosaccharides of known structure also showed that the antibody binds specifically to glycoconjugates with an alpha 1----3 terminal galactose residue. Anti-Gal did not bind to a human B-active ...
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GF ID He_PIG_assoc #=GF AC PF10632.8 #=GF DE He_PIG associated, NEW1 domain of bacterial glycohydrolase #=GF AU Naumoff D, Coggill P #=GF SE Pfam-B_97991 (release 22.0) #=GF GA 25.00 25.00; #=GF TC 25.20 30.80; #=GF NC 22.50 23.40; #=GF BM hmmbuild HMM.ann SEED.ann #=GF SM hmmsearch -Z 26740544 -E 1000 --cpu 4 HMM pfamseq #=GF TP Domain #=GF RN [1] #=GF RM 15285616 #=GF RT [Phylogenetic analysis of alpha-galactosidases of the GH27 #=GF RT family]. #=GF RA Naumov DG; #=GF RL Mol Biol (Mosk). 2004;38:463-476. #=GF RN [2] #=GF RM 16131397 #=GF RT GH97 is a new family of glycoside hydrolases, which is related #=GF RT to the alpha-galactosidase superfamily. #=GF RA Naumoff DG; #=GF RL BMC Genomics. 2005;6:112. #=GF DR INTERPRO; IPR019599; #=GF CC The English-language version of the first reference can be found #=GF CC on pages 388-399 of the above. This domain has been named NEW1 #=GF CC but its actual function is not known. It is found on proteins #=GF CC which are bacterial galactosidases [1]. The ...
Expression of N-terminally deleted beta-galactosidase.This protein and LacZ alpha-peptide (encoded by the pDrive Cloning Vector) provide alpha-galactosidase activity. Cells transformed by pDrive Cloning Vector which does not contain a PCR product will express LacZ alpha-peptide, and will form blue colonies when grown in the presence of X-gal/IPTG. In contrast, cells transformed by pDrive Cloning Vector which does contain a PCR product will not express LacZ alpha-peptide, and will form white colonies. ...
Learn about Fabrazyme: What is it used for, what you need to know before taking, important warnings and safety info, how to take, side effects and more...
Beano Drops information about active ingredients, pharmaceutical forms and doses by GlaxoSmithKline, Beano Drops indications, usages and related health products lists
In that case, lead plaintiff Joseph Carik, who has a doctors prescription for Fabrazyme, claimed: "By and through FDA consent, plaintiff has been banned interstate access to FDA-approved doses of Fabrazyme during a drug shortage created by Genzyme (a Sanofi company) an FDA licensee. Plaintiff is instead forcibly injected with a diluted, unregulated, unapproved dose of Fabrazyme because if the plaintiff refuses infusion of the unapproved dose, then the FDA licensee will withdraw any access and not provide future access to the drug until the shortage is over. The United States defendants have delegated all medical decisions during the shortage regarding plaintiffs disease, life, and health to the sole discretion and control of a corporation regulated by and through grant of an FDA license ...
Full Spectrum Digest is a full spectrum vegan enzyme blend, including Glutalytic, lactase, lipase, alpha-galactosidase, and amylase, to assist breakdown of gluten, gliadin, casein, whey, lactose, protein, fats, and carbohydrates.. ...
... -Full spectrum vegan enzyme blend, including Glutalytic®, lactase, lipase, alpha-galactosidase, and amylase, to assist breakdown of gluten, gliadin, casein, whey, lactose, protein, fats, and carbohydrates. Supplemen
HIGH PROTEIN & FAST DIGESTION: Zenith Whey delivers 26g protein out of 35g in each serving to help you get the foremost out of it. The all-time suitable for consumption protein is formulated with enzyme blend comprising Protease, Lactase, Lipase, Amylase, Alpha-galactosidase, Gluco-amylase, Invertase and Cellulase
[tabs][tab title=Ingredients]Plant Enzyme Blend 383 mg Amylase (3,500 DU) Glucoamylase (5.25 AGU) Lipase (350 FCCLU) Protease I pH 3.0 (15 S APU) Protease II pH 4.5 (20,000 HUT) Protease III pH 6.0 (35,000 HUT ) Cellulase (200 CU) CereCalaseT M (250 MU) Alpha-Galactosidase (75 GaIU) Lactase (150 ALU) Invertase (150
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FAGPL : Draw blood in a plain red-top tube(s), serum gel tube(s) is acceptable. Spin down and send 2 mL of serum refrigerated in a plastic vial.
Berechnung des absoluten Risiko in %, innerhalb von 10 Jahren ein tödliches Koronaereignis (z.B. Herzinfarkt) oder einen nicht-tödlichen Herzinfarkt zu erleiden.. ...
... My laboratory is investigating various aspects of how the immune system carries out surveillance to detect viral infections, cancers and cell death. Among the areas of research are: (1) The alarm signals and the receptors that alert the immune system to potential danger; (2) The mechanisms by which sentinel cells (dendritic cells) acquire and display antigens to CD8 T cells (cross presentation), a process that is essential for immune surveillance of tissues; and, (3) The antigen presentation pathway by which virally infected or cancer cells display their antigens to effector CD8 T cells (MHC class I antigen presentation), a process that is essential for the immune system to detect and eliminate these pathological cells. The laboratory is in a new state of the art research building and part of a very strong and interactive immunology community at UMass Medical School. UMass Medical School is located in Worcester Massachusetts, just outside of Boston. Interested ...
Background: Anderson-Fabry disease is a multisystem X linked disorder of lipid metabolism frequently associated with cardiac symptoms, including left ventricular (LV) hypertrophy gradually impairing cardiac function. Evidence showing that enzyme-replacement therapy (ERT) can be effective in reducing LV hypertrophy and improving myocardial function in the long term is limited. Objective: This study aimed to assess the long-term effects of ERT with recombinant α-galactosidase A (agalsidase beta, Fabrazyme) on LV function and myocardial signal intensity in 11 patients with Anderson-Fabry disease. Patients: Eleven patients (eight males, three females) with varying stages of genetically confirmed Anderson-Fabry disease were examined by means of physical examination and magnetic resonance imaging before ERT with agalsidase beta at 1 mg/kg every other week (study 1) and after a mean treatment duration of 45 months (study 2). Results: At 45 months of treatment, LV mass and LV wall thickness had ...
TY - JOUR. T1 - Urinary podocyte loss is increased in patients with fabry disease and correlates with clinical severity of fabry nephropathy. AU - Fall, Brent. AU - Scott, C. Ronald. AU - Mauer, Michael. AU - Shankland, Stuart. AU - Pippin, Jeffrey. AU - Jefferson, Jonathan A.. AU - Wallace, Eric. AU - Warnock, David. AU - Najafian, Behzad. PY - 2016/12. Y1 - 2016/12. N2 - Chronic kidney disease is a major complication of Fabry disease. Podocytes accumulate globotriaosylceramide inclusions more than other kidney cell types in Fabry patients. Podocyte injury occurs early in age, and is progressive. Since injured podocytes detach into the urine (podocyturia), we hypothesized that podocyturia would increase in Fabry patients and correlate with clinical severity of Fabry nephropathy. Urine specimens from 39 Fabry patients and 24 healthy subjects were evaluated for podocyturia. Most of the Fabry patients and many healthy subjects had podocyturia. The number of podocytes per gram of urine creatinine ...
Journal: EJNMMI - European Journal of Nuclear Medicine and Molecular Imaging ArticleTitle: Cardiac sympathetic neuronal damage precedes myocardial fibrosis in patients with Anderson-Fabry disease
People with Fabry Disease have an alteration in their genetic material (DNA) which causes a deficiency of the alpha-galactosidase A enzyme. Fabrazyme (agalsidase beta) is a drug that helps to break down and removes certain types of fatty substances called glycolipids. These glycolipids are normally present within the body in most cells. In Fabry disease, glycolipids build up in various tissues such as the liver, kidney, skin, and blood vessels because a-galactosidase A is not present, or is present in small quantities. The build up of glycolipid (globatriaosylceramide or GL-3) levels in these tissues in particular is thought to cause the clinical symptoms that are common to Fabry disease. This study analyzed the safety and efficacy of Fabrazyme in the treatment of patients with Fabry disease that previously participated in the AGAL-008-00 (NCT0074984) study ...
BACKGROUND: Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder caused by deficiency of alpha-galactosidase A. Central nervous system (CNS) manifestations consist mainly of cerebrovascular events. Brain MRI results are often abnormal. Purpose: The aim of the study was to describe CNS involvement in a group of Italian patients with AFD. METHODS: Clinical and brain MRI data of 43 patients with AFD (25 men, 41.94+/-10.83 years old and 18 women, 52.48+/-17.50 years old) were analysed retrospectively. 17 male patients and 7 female patients were under treatment with enzyme replacement therapy (ERT). RESULTS: All 43 patients had signs or symptoms of AFD. 16 men (64%) and 13 women (72%) demonstrated CNS involvement, although with varying severity. Overall, 6 men and 5 women had suffered from cerebrovascular accidents with an age at onset of 33.64+/-13.65 years and 53.68+/-11.71 years, respectively. Brain MR images were abnormal in 16/25 men and in 13/16 women. During CNS monitoring, ...
Release: Dec. 4, 2002. Childrens Hospital of Iowa to test new treatment for Fabry disease. The Childrens Hospital of Iowa at University of Iowa Hospitals and Clinics will be one of 20 medical centers to take part in a major international study of a new treatment for Fabry disease, a rare genetic disorder that affects an estimated 1 in 40,000 males worldwide. The goal of the trial is to determine the safety and efficacy of recombinant human alpha-galactosidase A (Fabrazyme) on the progression of renal disease and significant clinical events in patients with Fabry disease. This will be a Phase IV multi-center, multinational, randomized, double-blind, placebo-controlled trial. Because Fabry disease is a rare genetic disorder, centers will need to work aggressively to identify patients to take part in this study. We will be working with families, Fabry patient organizations and medical centers throughout our area to identify patients for this very important clinical trial. We are very pleased to ...
Background/Aims: Fabry disease is a treatable cause of chronic kidney disease (CKD) characterized by a genetic deficiency of a-galactosidase A. European Renal Best Practice (ERBP) recommends screening for Fabry disease in CKD patients. However, this is based on expert opinion and there are no reports of the prevalence of Fabry disease in stage 1-5 CKD. Hence, we investigated the prevalence of Fabry disease in CKD patients not receiving renal replacement therapy. Methods: This prospective study assessed a-galactosidase activity in dried blood spots in 313 stage 1-5 CKD patients, 167 males, between ages of 18-70 years whose etiology of CKD was unknown and were not receiving renal replacement therapy. The diagnosis was confirmed by GLA gene mutation analysis. Results: Three (all males) of 313 CKD patients (0.95%) were diagnosed of Fabry disease, for a prevalence in males of 1.80%. Family screening identified 8 aditional Fabry patients with CKD. Of a total of 11 Fabry patients, 7 were male and ...
Introduction. Fabry disease is a rare genetic lysosomal storage disorder of glycosphingolipids with X-linked transmission and an estimated incidence of 1:40,000-1:117,000 live male births.1 Partial or complete deficiency of the enzyme alpha-galactosidase A (a-Gal A) results in altered metabolism and progressive lysosomal accumulation of the substrate (mostly globotriaosylceramide, Gb3).2 The responsible gene is located on the long arm of the chromosome X (Xq22). More than 600 mutations have been identified with variable phenotypical expression.3. Clinically we distinguish the classical form and two variants, cardiac and renal. In the classical form clinical manifestations appear during childhood or early adolescence including acroparesthesias, angiokeratomas and corneal opacities.4 Progressive accumulation of Gb3 in the kidneys, heart and central nervous system lead to renal failure, hypertrophic cardiomyopathy and cerebral vascular accidents limiting life expectancy. The cardiac variant of the ...
Anderson Fabry disease (AFD) is an X-linked lysosomal storage disorder caused by a deficiency of the enzyme alpha-galactosidase. AFD can involve various organs and lead to a series of clinical abnormalities. Left ventricular hypertrophy in middle-aged men is one of its life threatening complications. It was shown that pending the absence of myocardial replacement fibrosis, substitution therapy could improve myocardial morphology and function as well as exercise capacity. Today, there is no available marker of the efficacy of the treatment on the heart morphology and function.. The T1 time (or longitudinal relaxation time) is one of the major components of the image formation in Magnetic Resonance Imaging (along with T2 time and proton density). Several techniques have been described to assess the myocardial T1-time.. One of them called MOLLI (Modified Look Locker Inversion Recovery), was made available in research centres by the Siemens company. In a study published in 2013, Sado et al. showed ...
Fabry disease is a rare X-linked hereditary disease caused by mutations in the AGAL gene encoding the lysosomal enzyme alpha-galactosidase A. Enzyme replacement therapy (ERT) is the current cornerstone of Fabry disease management. Involvement of kidney, heart and the central nervous system shortens life span, and fibrosis of these organs is a hallmark of the disease. Fibrosis was initially thought to result from tissue ischemia secondary to endothelial accumulation of glycosphingolipids in the microvasculature. However, despite ready clearance of endothelial deposits, ERT is less effective in patients who have already developed fibrosis. Several potential explanations of this clinical observation may impact on the future management of Fabry disease. Alternative molecular pathways linking glycosphingolipids and fibrosis may be operative; tissue injury may recruit secondary molecular mediators of fibrosis that are unresponsive to ERT, or fibrosis may represent irreversible tissue injury that ...
In this paper, two cases of renal biopsy showing identical lipid deposits were presented. In the first case, these lipid deposits were due to inhibition of intralysosomal alpha-galactosidase A activity (presumable hydroxychloroquine-induced renal phospholipidosis) and in the second case, due to a mutation in the gene encoding the referred enzyme.. FD is a X-chromosome linked genetic disorder characterized by disturbance in glycosphingolipid catabolism, caused by a deficiency of the enzyme alpha-galactosidase A. Early signs and symptoms occur from childhood to adolescence and include intermittent paresthesia and acroparesthesia, "Fabry crisis" (episodes of intense pain), recurrent fever, angiokeratomas, cornea verticillata, mild proteinuria, globotriaosylceramide in urinary sediment and digestive symptoms such as both diarrhea and constipation, nausea, vomiting and abdominal cramps. Manifestations in adolescence and adulthood include renal disorders (which can progress to end-stage kidney ...
Fabrys is an X-linked lysosomal storage disorder caused by a deficiency in the enzyme alpha-galactosidase A. As a result, a buildup of glycosphyngolipids in cellular lysosomes occurs, a process thought to lead to cellular dysfunction. The effects are particularly seen in the nervous system, causing painful acroparesthesias; cardiac myocytes, leading to hypertrophic cardiomyopathy; and the kidneys, where endothelial storage of glycosphingolipids leads to vascular insufficiency and glomerular damage. A typical affected male will manifest frequent episodes of burning limb pain starting in childhood, which unfortunately respond poorly to analgesics. Other early signs of Fabrys include gastrointestinal pain and hypohydrosis, due to involvement of nerves innervating the gut and skin. Angiokeratomas, small reddish-purple lesions characteristic of the disease, are often present, especially in the groin area (and are often missed on physical examination).. Cardiac and renal involvement usually manifest ...
Fabry disease is an X-linked lysosomal storage disorder that leads to excessive deposition of globotriaosylceramide ( GL-3) throughout the body. Skin, eye, kidney, heart, brain, and peripheral nervous system are highly vulnerable. Fabry disease is often difficult to diagnose since signs and symptoms are often nonspecific.. Symptoms. Many symptoms associated with Fabry disease are nonspecific making it a difficult disease to diagnosis. Not all symptoms may appear nor develop in any particular order. However, younger patients may have some or all of the following:. ...
Allcosmeticsource.com alpha galactosidase 2000u/g,5kg/bag,free shipping [EP170508006]- alpha galactosidase 2000u/g,1kg/bag,free shipping What is alpha galactosidase 2000u/g alpha galactosidase is an enzyme used to hydrolyze or break α-1, 6-glycosidic bonds into galactosyl oligosaccharides (α-galactosides), liberating simpler, more usable sugars and eliminating its anti-nutrient effect. Thus, it improves the utilization of the energy and proteins in feedstuff. Function of alpha galactosidase 2000u/g Hydrolyzing
Patients affected by a rare and potentially fatal inherited metabolic disorder called Fabry disease are now benefiting from a new treatment that researchers at Childrens Hospital of Iowa helped create.. Fabry disease affects approximately 5,000 people worldwide. The average lifespan of people diagnosed with the disorder is 50 years. The disease is caused by a deficiency of an enzyme called alpha-galactosidase A. Patients affected by the condition can develop kidney failure, strokes, heart disease and disabling pain. The Food and Drug Administration (FDA) recently approved a medication called Fabrazyme, produced by Genzyme General, for the treatment of Fabry disease. The therapy replaces the missing enzyme in order to help correct the disorder. The treatment took 30 years of research to develop.. Thomas Loew, M.D., associate professor (clinical) at Childrens Hospital of Iowa in University of Iowa Hospitals and Clinics, helped conduct the research that led to the FDAs approval of ...
Background: Fabry disease. an X-linked deficiency of α-galactosidase A coded by the GLA gene, leads to intracellular globotriaosylceramide (GL-3) accumulation. Although less common than in males, chronic kidney disease, occurs in ~15% of females. Recent studies highlight the importance of podocyte injury in Fabry nephropathy development and progression. We hypothesized that the greater the % of podocytes with active wild-type GLA gene (due to X-inactivation of the mutant copy) the less is the overall podocyte injury. Methods: Kidney biopsies from 12 treatment-naive females with Fabry disease, ages 15 (8-63), median [range], years were studied by electron microscopy and compared with 4 treatment-naive male patients.. Results: In females, 51 (13-100)% of podocytes (PC) per glomerulus had no GL-3 inclusions, this consistent with a non-Fabry podocyte phenotype (NFPC). In PC with GL-3 inclusions [Fabry podocyte phenotype (FPC)], GL-3 volume density per podocyte was virtually identical in females and ...
Enzyme replacement therapy can reduce storage, ease pain, and preserve organ function in some individuals with Fabry disease. Drugs are often prescribed to treat pain that accompanies Fabry disease but does not treat the disorder. The U.S. Food and Drug Administration has approved migalastat (Galafold) as an oral medication for adults with Fabry disease who have a certain genetic mutation. Anti-platelet medications can help prevent strokes and medications that lower blood pressure can slow the decline of kidney function in people with Fabry disease. ...
Cardiac microvascular function abnormalities in Fabry patients have been demonstrated by measurements of myocardial blood flow and coronary flow reserve, an index of microvascular function.14 In addition, a survey of female Fabry patients revealed that cardiac ischemia could be confirmed by ECG and serological markers in the absence of coronary artery stenosis, suggesting that the ischemia in these patients was of microvascular origin.11 However, widespread coronary artery disease also has been documented in Fabry patients15 and noted at autopsy in a male patient who died of a massive myocardial infarction.16 In the present study, we characterized the baseline pathology of GL-3 accumulation in cardiac biopsies and demonstrated its successful clearance from the microvasculature after enzyme replacement therapy.. It has long been established that GL-3 is transported in low- and high-density lipoprotein particles17-19 and that vascular cells accumulate GL-3 from the circulation through the ...
Fabry disease is a rare enzyme deficiency known as a lysosomal storage disease. Wikipedia The enzyme involved, alpha galactosidase A, is coded by the GLA gene. OMIM Although Fabry disease has been considered an X-linked recessive condition, female carriers of a single mutated GLA gene may have significant symptoms. Enzyme replacement therapy is helpful, although it is currently extremely expensive, and production problems have led to shortages of the drug. [1] ...
Clark, NE, Garman, SC. The 1.9 A structure of human alpha-N-acetylgalactosaminidase: The structural basis of Schindler and Kanzaki diseases. Journal of Molecular Biology. 2009, Oct 23;393(2):435-447. [PubMed]. Ishii S, Chang HH, Kawasaki K, Yasuda K, Wu HL, Garman SC, Fan JQ. Mutant alpha-galactosidase A enzymes identified in Fabry disease patients with residual enzyme activity: biochemical characterization and restoration of normal intracellular processing by 1-deoxygalactonojirimycin. Biochem J. 2007 Sep 1;406(2):285-95.[PubMed]. Hebert DN, Garman SC, Molinari M. (2005) "The glycan code of the endoplasmic reticulum: asparagine-linked carbohydrates as protein maturation and quality-control tags." Trends Cell Biol. Jul;15(7):364-70. Review.[PubMed]. Su HP, Garman SC, Allison TJ, Fogg C, Moss B, Garboczi DN. (2005) "The 1.51-Angstrom structure of the poxvirus L1 protein, a target of potent neutralizing antibodies." Proc Natl Acad Sci U S A. Mar 22;102(12):4240-5. Epub 2005 Mar 10. [PubMed]. Ries ...
In this article, alpha-galactosidase A activity was assayed in newborn screening blood spots of Italian male neonates. This study revealed a high incidence of later-onset Fabry disease.. For more information on Fabry disease, see chapter 150 of OMMBID ...
Figure 1. Cardiac hypertrophy in a patient with Fabry disease. A. and B. Concentric LV hypertrophy. Note the presence of a hyperechogenic region in the posterior wall (mid-wall level, arrow), corresponding to localized fibrosis. C. Right ventricular free wall hypertrophy from subcostal view.. In most cases of FD, the LVH is concentric and non-obstructive; however, an asymmetrical hypertrophy with septal thickening and posterior wall fibrotic thinning may present in advanced cases. There have also been rare case reports of patients with obstructive forms of hypertrophic cardiomyopathy. The LVH is progressive in nature, and is rarely severe in children or adolescents. Of note, the echocardiographically derived cardiac mass is proportional to the electrocardiographic LVH low-voltage on the ECG, presenting an argument against Fabry disease in these patients.. Right ventricular (RV) hypertrophy is also common [9] (around 70% of Fabry patients display it) and may progress to RV dilation (Figure 1C). ...
BACKGROUND: Fabry disease (FD) is an X-linked lysosomal storage disorder frequently associated with cerebrovascular disease. In recent years, the prevalence of FD has been reported to be up to 4% in cryptogenic young stroke patients. However, there have been no population-based studies in unselected patients with transient ischaemic attack (TIA) or stroke across the full range of ages. METHODS: We determined the prevalence of FD mutations in consecutive patients from a population-based study of acute TIA or ischaemic stroke (Oxford Vascular Study). Analysis included amplifying of the α-galactosidase A gene by polymerase chain reaction, denaturing high-performance liquid chromatography (dHPLC) analysis and sequencing using standard automated sequencing protocols [Mutation Surveyor software (Softgenetics)] where the dHPLC indicated a possible mutation. RESULTS: Samples of 1046 consecutive patients (52% women; mean age 73.2 years; 15% age |60 years; 572 stroke; 474 TIA) were tested. No patient had a known
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The European Medicines Agency (EMA) has recommended granting a marketing authorisation in the European Union (EU) for migalastat (Galafold) for the treatment of Fabry disease, a rare genetic disorder. Patients with Fabry disease do not have enough of an enzyme called alpha-galactosidase A.
6-phospho-alpha-glucosidase (EC 3.2.1.122); alpha-galactosidase (EC 3.2.1.22). 6-phospho-alpha-glucosidase requires both NAD(H ... dependent 6-phospho-alpha-glucosidase. Assignment to family 4 of the glycosylhydrolase superfamily". J. Biol. Chem. 273 (42): ...
"Molecular basis of beta-galactosidase alpha-complementation". Proceedings of the National Academy of Sciences of the United ... β-galactosidase is a protein encoded by the lacZ gene of the lac operon, and it exists as a homotetramer in its active state. ... The presence of an active β-galactosidase can be detected by X-gal, a colourless analog of lactose that may be cleaved by β- ... However, a mutant β-galactosidase derived from the M15 strain of E. coli has its N-terminal residues 11-41 deleted and this ...
Alpha-galactosidase is present in a variety of organisms. There is a considerable degree of similarity in the sequence of alpha ... a superfamily of alpha-galactosidases, alpha-N-acetylgalactosaminidases, and isomaltodextranases which are likely to share a ... contains a region of about 50 amino acids which is similar to a domain of the eukaryotic alpha-galactosidases. Alpha-N- ... galactosidase from various eukaryotic species. Escherichia coli alpha-galactosidase (gene melA), which requires NAD and ...
... alpha-galactosidase (EC 3.2.1.22); glucoamylase (EC 3.2.1.3), sucrase-isomaltase (EC 3.2.1.48) (EC 3.2.1.10); alpha-xylosidase ... van Beeumen J, Kroos MA, Oostra BA, Hermans MM, Reuser AJ (1991). "Human lysosomal alpha-glucosidase. Characterization of the ... Glycoside hydrolase family 31 CAZY GH_31 comprises enzymes with several known activities; alpha-glucosidase (EC 3.2.1.20), ... Homology with the rabbit intestinal sucrase-isomaltase complex and human lysosomal alpha-glucosidase". Eur. J. Biochem. 202 (2 ...
It contains the enzymes alpha-galactosidase (α-GAL) and invertase. It was introduced as a liquid, but that has been ... "The effect of oral alpha-galactosidase on intestinal gas production and gas-related symptoms". Dig. Dis. Sci. 52 (1): 78-83. ... A double-blind crossover study of oral alpha-galactosidase to treat dietary oligosaccharide intolerance". J Fam Pract. 39 (5): ...
It is metabolized by alpha-galactosidase, which hydrolyzes the terminal alpha linkage. Defects in the enzyme alpha- ... The pharmaceutical drug migalastat enhances the function of alpha-galactosidase and is used to treat Fabry's. ... a-Galactosidase A deficiency: Fabry disease. In: Scriver CR, Beaudet AL, Sly WS, Valle D, eds. The metabolic & molecular bases ... It is formed by the alpha linkage of galactose to lactosylceramide catalyzed by A4GALT. ...
Fabrazyme (agalsidase beta, or Alpha-galactosidase) was licensed to the Genzyme Corporation. It is an enzyme replacement ... A deficiency of the enzyme alpha galactosidase A (a-GAL A, encoded by GLA) due to mutation causes a glycolipid known as ... to measure the level of alpha-galactosidase activity. An enzyme assay is not reliable for the diagnosis of disease in females ... The pharmaceutical company Shire manufactures agalsidase alpha (which differs in the structure of its oligosaccharide side ...
doi:10.1007/s12257-010-0069-0. Dey PM1, Patel S, Brownleader MD (June 1993). "Induction of alpha-galactosidase in Penicillium ...
... pegfilgrastim sold as Neulasta alpha-galactosidase A: Fabrazyme by Genzyme alpha-L-iduronidase: (rhIDU; laronidase) Aldurazyme ...
The enzyme alpha-galactosidase A (α-GalA) Globotriaosylceramide (Gb3), a substrate of α-GalA, has a terminal D-galactose ... "The pharmacological chaperone 1-deoxygalactonojirimycin increases alpha-galactosidase A levels in Fabry patient cell lines". ... of Fabry disease in adults and adolescents aged 16 or older with an amenable mutation of the enzyme alpha-galactosidase A (α- ... ISBN 0-470-03391-6. Miyake, Y; Ebata, M (1988). "The structures of a β-galactosidase inhibitor, Galactostatin, and its ...
Anisha, GS; Sukumaran, RK; Prema, P (March 2008). "Evaluation of alpha-galactosidase biosynthesis by Streptomyces griseoloalbus ... Anisha, G. S.; John, Rojan P.; Prema, P.; Pandey, Ashok (4 September 2008). "Investigation on α-Galactosidase Production by ... Anisha, G. S.; Prema, P. (5 December 2006). "Production of α-galactosidase by a novel actinomycete Streptomyces griseoloalbus ... "Production and characterization of partially purified thermostable α-galactosidases from Streptomyces griseoloalbus for food ...
The non-catalytic domains of glycosidases from the alpha-galactosidase and alpha-glucosidase superfamilies are also ... Naumoff DG (2005). "GH97 is a new family of glycoside hydrolases, which is related to the alpha-galactosidase superfamily". BMC ... In all known glycosidases with the (beta-alpha)8-barrel fold, the amino acid residues at the active site are located on the C- ... The central part of the GH97 family protein sequences represents a typical and complete (beta/alpha)8-barrel or catalytic TIM- ...
... α-galactosidase (EC 3.2.1.22); amylopullulanase (EC 3.2.1.41); branching enzyme (EC 2.4.1.18). It includes a thermostable alpha ... Glycoside hydrolase family 57 CAZY GH_57 comprises enzymes with several known activities; alpha-amylase (EC 3.2.1.1), 4-alpha- ... 1993). "The purification and characterization of an extremely thermostable alpha-amylase from the hyperthermophilic ...
Supplements of the enzyme supplement alpha-galactosidase may reduce symptoms (if brands containing other FODMAPs are avoided). ...
Cloning and expression of biologically active alpha-galactosidase A as a fusion protein". "USPTO: Cloning and expression of ... biologically active alpha N-acetylgalactosaminidase". "USPTO: Cloning and expression of biologically active alpha-galactosidase ... "Cloning and expression of biologically active human alpha-galactosidase A". The Mount Sinai Hospital homepage Icahn School of ... National Institute of Diabetes and Digestive and Kidney Diseases Alpha Galactosidases A And B -- Molecular and Cellular ...
... contains an alpha-galactosidase enzyme; this enzyme, which hydrolyses the terminal alpha-galactosyl moieties from glycolipids ... Cai GL, Lu J (2012). "Isolation and identification of a novel Aspergillus sydowii F5 Producing alpha-galactosidase and ... is used in enzyme replacement therapy to functionally compensate for genetic alpha-galactosidase deficiency. "Aspergillus ...
... ceramidetrihexoside alpha-galactosidase, trihexosylceramide alpha-galactosidase, and ceramidetrihexosidase. Brady RO, Gal AE, ... Other names in common use include trihexosyl ceramide galactosidase, ceramide trihexosidase, ...
Other genetically modified pigs have had alpha galactosidase transferase knocked out and fortified with hCD46 and the hTM ... Human-alpha-1-antitrypsin, which has been tested in sheep and is used in treating humans with this deficiency and transgenic ... These include alpha-amylase from bacteria, which converts starch to simple sugars, chymosin from bacteria or fungi, which clots ... "Transgenic pigs designed to express human α-galactosidase to avoid humoral xenograft rejection". J Appl Genet. 54 (3): 293-303 ...
1995). "Sixty-nine kilobases of contiguous human genomic sequence containing the alpha-galactosidase A and Bruton's tyrosine ...
It contains the enzymes alpha-galactosidase (α-GAL) and invertase. It was introduced as a liquid, but that has been ... Di Stefano M, Miceli E, Gotti S, Missanelli A, Mazzoccahi S, Corazza GR (January 2007). "The effect of oral alpha-galactosidase ... A double-blind crossover study of oral alpha-galactosidase to treat dietary oligosaccharide intolerance". J Fam Pract. 39 (5): ...
... plant cell culture expressed and a chemically modified version of the recombinant alpha-Galactosidase-A protein. Protein sub- ...
Alpha-galactosidase, an enzyme that breaks down certain complex sugars, is a component of Beano and other products that ...
1989). "Molecular cloning of a full-length cDNA for human alpha-N-acetylgalactosaminidase (alpha-galactosidase B)". Biochem. ... Homology with human alpha-galactosidase A suggests evolution from a common ancestral gene". J. Biol. Chem. 265 (35): 21859-66. ... Warner TG, Louie A, Potier M (1991). "Photolabeling of the alpha-neuraminidase/beta-galactosidase complex from human placenta ... 1996). "Human alpha-N-acetylgalactosaminidase (alpha-NAGA) deficiency: new mutations and the paradox between genotype and ...
The enzyme alpha-galactosidase now is allowed to be produced in moss bioreactors by the German Federal Institute for Drugs and ...
Some species of mold produce alpha-galactosidase, an anti-oligosaccharide enzyme, which humans can take to facilitate digestion ...
Beta-galactosidase. *Hexosaminidase. *mannosidase *alpha-Mannosidase. *beta-mannosidase. *Aspartylglucosaminidase. *Fucosidase ...
Beta-galactosidase Migalastat, a drug targeting alpha-galactosidase Classification of α-galactosidases (according to CAZy) ... Dean KJ, Sweeley CC (1979). "Studies on human liver alpha-galactosidases. I. Purification of alpha-galactosidase A and its ... Alpha-galactosidase is a glycoside hydrolase enzyme that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and ... Two recombinant forms of alpha-galactosidase are called agalsidase alpha (INN) and agalsidase beta (INN). This enzyme is a ...
Detailed Alpha-D-Galactosidase dosage information for adults. Includes dosages for Flatulence; plus renal, liver and dialysis ... Alpha-D-galactosidase oral drops:. Take 5 drops/problem food right before your first bite. A typical meal has 3 servings of ... Alpha-D-galactosidase oral tablet/capsule, chewable:. Chew or swallow whole 1 tablet/capsule per problem food right before your ...
... alpha-galactosidase A (alpha-Gal A; alpha-D-galactoside galactohydrolase, EC 3.2.1.22). To investigate the structure, ... Fabry disease: isolation of a cDNA clone encoding human alpha-galactosidase A. D H Calhoun, D F Bishop, H S Bernstein, M Quinn ... Fabry disease: isolation of a cDNA clone encoding human alpha-galactosidase A ... Fabry disease: isolation of a cDNA clone encoding human alpha-galactosidase A ...
Drops; Oral; Alpha-Galactosidase 150 galu / 5 drop*Tablets; Oral; Alpha-Galactosidase 13.8 mg. ...
Capsular-polysaccharide endo-1,3-alpha-galactosidase at the US National Library of Medicine Medical Subject Headings (MeSH) ... Capsular-polysaccharide endo-1,3-alpha-galactosidase (EC 3.2.1.87, polysaccharide depolymerase, capsular polysaccharide ... alpha-D-galactosidic linkages in Aerobacter aerogenes capsular polysaccharide Hydrolyses the galactosyl-alpha-1,3-D-galactose ...
Alpha-Galactosidase. Available forms, composition and doses of Beano Drops:. *Drops; Oral; Alpha-Galactosidase 150 galu / 5 ...
tr,Q02402,Q02402_9FUNG Alpha-galactosidase OS=Umbelopsis vinacea OX=44442 GN=alpha-galactosidase PE=2 SV=1 ... Name:alpha-galactosidaseImported. ,p>Information which has been imported from another database using automatic procedures.,/p ... Hydrolysis of terminal, non-reducing alpha-D-galactose residues in alpha-D-galactosides, including galactose oligosaccharides, ...
An improved beta-galactosidase alpha-complementation system for molecular cloning in Bacillus subtilis.. Haima P1, van Sinderen ... The recently described beta-galactosidase alpha-complementation system for molecular cloning in Bacillus subtilis [Haima et al ... It was shown that large heterologous DNA fragments (up to at least 29 kb) could be cloned with lacZ alpha-complementing vectors ... Third, a new lacZ alpha complementing cloning vector was constructed, containing more unique target sites. ...
Wikipedia on Alpha-galactosidase deficiency (Less technical, ? quality control). UpToDate® on Alpha-galactosidase deficiency ( ... Alpha-galactosidase deficiency (Fabry disease, Fabry syndrome, Anderson-Fabry syndrome) is caused by gene variations that ... Nice Guidance on Alpha-galactosidase deficiency. Centre for Reviews and Dissemination databases -DARE & NHS EED (evaluates ... Agalsidase alpha (may not be as effective from switch studies[3]) *Pharmacological chaperones may help with specific mutations ...
Human lysosomal enzymes acid-beta-glucosidase (GCase) and acid-alpha-galactosidase (alpha-Gal A) hydrolyze the sphingolipids ... Human lysosomal enzymes acid-beta-glucosidase (GCase) and acid-alpha-galactosidase (alpha-Gal A) hydrolyze the sphingolipids ... Crystal structure of alpha-galactosidase A at pH 4.5 complexed with 1-deoxygalactonijirimycin. *DOI: 10.2210/pdb3GXT/pdb ... including the first structure of alpha-Gal A with DGJ. Both GCase and alpha-Gal A are more stable at lysosomal pH with and ...
Analysis of splice-site mutations of the alpha-galactosidase A gene in Fabry disease.. Lai LW1, Whitehair O, Wu MJ, OMeara M, ... Fabry disease is an X-linked disease caused by a defective lysosomal enzyme, alpha-galactosidase A, and characterized by skin ...
Human lysosomal enzymes acid-beta-glucosidase (GCase) and acid-alpha-galactosidase (alpha-Gal A) hydrolyze the sphingolipids ... Human lysosomal enzymes acid-beta-glucosidase (GCase) and acid-alpha-galactosidase (alpha-Gal A) hydrolyze the sphingolipids ... Alpha-galactosidase A. A, B. 398. Homo sapiens. Mutation(s): 0 Gene Names: GLA. EC: 3.2.1.22 (PDB Primary Data), 3.2.1.47 ( ... Crystal structure of acid-alpha-galactosidase A complexed with galactose at pH 4.5. *DOI: 10.2210/pdb3GXP/pdb ...
... alpha-Gal A), resulting in renal failure along with premature myocardial infarction and strokes. No effective treatment of this ... Fabry disease is a disorder of glycosphingolipid metabolism caused by deficiency of lysosomal alpha-galactosidase A ( ... Fabry disease is a disorder of glycosphingolipid metabolism caused by deficiency of lysosomal alpha-galactosidase A (alpha-Gal ... Accelerated transport and maturation of lysosomal alpha-galactosidase A in Fabry lymphoblasts by an enzyme inhibitor Nat Med. ...
The enzyme alpha-galactosidase (alpha-GAL, also known as alpha-GAL A; E.C. 3.2.1.22) is responsible for the breakdown of alpha- ... a lysosomal storage disorder characterized by the buildup of alpha-galactosylated substrates in the tissues. alpha-GAL is an ... The high resolution structures of each step in the catalytic cycle will allow for improved drug design efforts on alpha-GAL and ... Defects in human alpha-GAL lead to the development of Fabry disease, ...
Clinical characteristics of patients with alpha-galactosidase A gene variants in a German multicentre cohort of early ... Clinical characteristics of patients with alpha-galactosidase A gene variants in a German multicentre cohort of early ...
The addition of 50 mM glucose did not affect the induction of alpha-galactosidase formation by galactose. alpha-Galactosidase ... Conditions of formation, purification, and characterization of an alpha-galactosidase of Trichoderma reesei RUT C-30.. S ... Trichoderma reesei RUT C-30 formed an extracellular alpha-galactosidase when it was grown in a batch culture containing lactose ... Conditions of formation, purification, and characterization of an alpha-galactosidase of Trichoderma reesei RUT C-30. ...
J. Med. 276, 1163 (1967). Identification as an a-galactosidase: J. A. Kint, Science 167, 1268 (1970). Use in enzyme replacement ... Literature References: Human a-galactosidase A produced by recombinant DNA technology in Chinese hamster ovary cells. See: R. J ... Literature References: Human a-galactosidase A produced by recombinant DNA technology in cultured human cells. See: R. F. ... CAS Name: a-Galactosidase (human clone lAG18 isoenzyme A subunit protein moiety reduced) ...
Alpha galactosidase A Antibody 19877-1-AP has been identified with IF, WB, ELISA. 19877-1-AP detected 49 kDa band in HEK-293 ... Agalsidas and Alpha-galactosidase A, Belongs to the glycosyl hydrolase 27 family. It hydrolysis of terminal, non-reducing alpha ... Alpha galactosidase A Antibody 0 Publications. Rabbit Polyclonal, Catalog number: 19877-1-AP ... HEK-293 cells were subjected to SDS PAGE followed by western blot with 19877-1-AP(Alpha galactosidase A antibody) at dilution ...
Alpha galactosidase A Antibody 66121-1-Ig has been identified with IF, IHC, WB, ELISA. 66121-1-Ig detected 49 kDa band in HeLa ... Agalsidase and Alpha-galactosidase A, belongs to the glycosyl hydrolase 27 family. It hydrolyzes terminal, non-reducing alpha-D ... Alpha galactosidase A Antibody 0 Publications. Mouse Monoclonal, Catalog number: 66121-1-Ig ,CloneNo.: 2B2C5 Featured Product ... HeLa cells were subjected to SDS PAGE followed by western blot with 66121-1-Ig(Alpha galactosidase A antibody) at dilution of 1 ...
H1 2016 Alpha-Galactosidase A (Alpha-D-Galactosidase A or Alpha-D-Galactoside Galactohydrolase or - Market research report and ... Alpha-D-Galactosidase A or Alpha-D-Galactoside Galactohydrolase or Melibiase or EC 3.2.1.22) - Pipeline Review, ... Alpha-Galactosidase A (Alpha-D-Galactosidase A or Alpha-D-Galactoside Galactohydrolase or Melibiase or EC 3.2.1.22) - Pipeline ... Alpha-Galactosidase A (Alpha-D-Galactosidase A or Alpha-D-Galactoside Galactohydrolase or Melibiase or EC 3.2.1.22) Overview ...
Recombinant Protein and Alpha-galactosidase Antibody at MyBioSource. Custom ELISA Kit, Recombinant Protein and Antibody are ... Alpha-galactosidase A. Alpha-galactosidase A ELISA Kit. Alpha-galactosidase A Recombinant. Alpha-galactosidase A Antibody. Also ... Alpha-galactosidase C. Alpha-galactosidase C ELISA Kit. Alpha-galactosidase C Recombinant. Alpha-galactosidase C Antibody. Also ... Alpha-galactosidase D. Alpha-galactosidase D ELISA Kit. Alpha-galactosidase D Recombinant. Alpha-galactosidase D Antibody. Also ...
3-galactosidase B Recombinant Protein-WP_005843041.1 (MBS1078671) product datasheet at MyBioSource, Recombinant Proteins ... Alpha-galactosidase. Roves both branched alpha-1,3-linked galactose residues of blood group B antigens and linear alpha-1,3- ... Alpha-galactosidase. Removes both branched alpha-1,3-linked galactose residues of blood group B antigens and linear alpha-1,3- ... Alpha-1,3-galactosidase B (glaB1), Recombinant Protein. ★Popular Item★ Also Known As Recombinant Bacteroides vulgatus Alpha-1,3 ...
Fabry disease is caused by mutations in the gene encoding the lysosomal hydrolase, α-Gal A. The galactosidase, alpha (GLA) gene ... Eng, CM, Guffon, N, Wilcox, WR, Germain, DP, Lee, P, Waldek, S. "Safety and efficacy of recombinant human alpha-galactosidase A ... Fabry Disease (Anderson-Fabry Disease, Alpha-Galactosidase A Deficiency, Angiokeratoma Corporis Diffusum, Ceramide ... Fabry Disease (Anderson-Fabry Disease, Alpha-Galactosidase A Deficiency, Angiokeratoma Corporis Diffusum, Ceramide ...
... , digestive enzymes, enzymes, FODMAPs, fructose intolerance, glucose isomerase, lactase, xylose isomerase ...
Physical and kinetic properties of alpha-galactosidase A in cultured human endothelial cells. by Douglas L. Johnson et al. ... Physical and kinetic properties of alpha-galactosidase A in cultured human endothelial cells.. *. Douglas L. Johnson. , Robert ... Physical and kinetic properties of alpha-galactosidase A in cultured human endothelial cells.}, author={Douglas L. Johnson and ... Lysosomal α-galactosidase in endothelial cell cultures established from a fabry hemizygous and normal umbilical veins. *Lis ...
  • Short-chain alpha-galactosides (melibiose, raffinose, stachyose), as well as the monosaccharides galactose, dulcitol, arabinose, and arabitol, also induced alpha-galactosidase activity both when they were used as carbon sources (at a concentration of 1%) in batch cultures and in resting mycelia (at concentrations in the millimolar range). (asm.org)
  • Studies of residual activities of mutant enzymes in many Fabry patients showed that some of them had kinetic properties similar to those for normal alpha-Gal A, but were significantly less stable, especially in conditions of neutral pH (refs. (nih.gov)
  • Alpha-amylases are digestive enzymes which hydrolyze glycosidic bonds in starch to glucose, maltose, malt triose and dextrin. (alpspure.co.in)
  • The formula supplies the added support of alpha-galactosidase and beta-glucanase to break down vegetables, beans and grains, and enzymes such as glucoamylase and invertase to assist with the digestion of sugars. (iherb.com)
  • The characterization and properties of a beta-galactanase and alpha- and beta-galactosidases as well as heparan sulfate and chondroitin sulfate degrading enzymes which appear during the 15 days of the embryonic development of the mollusc Pomacea sp. (unifesp.br)
  • In addition, immediate anaphylaxis may develop after exposure to other sources of alpha-gal, such as monoclonal antibody cetuximab, vaccines, plasma expanders or anti-snake venoms. (elsevier.es)
  • Inhibition of alpha galactosidase was seen by N-Bromosuccinamide, oxidation of two tryptophan residues, Modification of thyrosine residue and carbodiamide. (who.int)
  • showed that the mutant non-functional β-galactosidase was lacking in part of its N-terminus with its residues 11-41 deleted, but it may be complemented by a peptide formed of residues 3-90 of β-galactosidase. (wikipedia.org)
  • However, a mutant β-galactosidase derived from the M15 strain of E. coli has its N-terminal residues 11-41 deleted and this mutant, the ω-peptide, is unable to form a tetramer and is inactive. (wikipedia.org)
  • In this method of screening, the host E. coli strain carries the lacZ deletion mutant ( lacZΔM15 ) which contains the ω-peptide, while the plasmids used carry the lacZα sequence which encodes the first 59 residues of β-galactosidase, the α-peptide. (wikipedia.org)
  • This paper reports on the effects of both reducing and nonreducing transgalactooligosaccharides (TOS) comprising 2 to 8 residues on the growth of Bifidobacterium adolescentis DSM 20083 and on the production of a novel β-galactosidase (β-Gal II). (asm.org)
  • An alpha-N-acetylglucosaminidase and a beta-glucuronidase which act upon the N-acetylated fragments formed from heparan sulfate emerge around day 4 of development. (unifesp.br)
  • This modulation might have profound impact on the clinical outcome of Fabry patients treated with rh alpha-GalA. (unifesp.br)
  • The present study describes the clinical and sensitization characteristics of 39 patients diagnosed with alpha-gal allergy in the hospitals of our province (Lugo, Monforte de Lemos and Burela, Spain). (elsevier.es)
  • Conclusions- The findings suggest that long-term treatment with recombinant human α-galactosidase A may halt the progression of vascular pathology and prevent the clinical manifestations of atherosclerotic disease. (ahajournals.org)
  • alpha galactosidase is an enzyme used to hydrolyze or break α-1, 6-glycosidic bonds into galactosyl oligosaccharides (α-galactosides), liberating simpler, more usable sugars and eliminating its anti-nutrient effect. (allcosmeticsource.com)
  • Methods and Results- We analyzed pretreatment and posttreatment endomyocardial biopsies from 58 Fabry patients enrolled in a 5-month, phase 3, double-blind, randomized, placebo-controlled trial, followed by a 54-month open-label extension study of recombinant human α-galactosidase A. Baseline evaluations revealed GL-3 deposits in interstitial capillary endothelial cells and large, laminated inclusions within cardiomyocytes. (ahajournals.org)
  • We have previously reported the results of enzyme replacement therapy with recombinant human α-galactosidase A (r-hαGalA) on the histological clearance of GL-3 from both renal 1 and dermatologic biopsies 2 obtained during a phase 3 trial. (ahajournals.org)
  • Purified alpha galactosidase was reduced alkylated, and fragment. (who.int)
  • This phenomenon of α-complementation was first demonstrated in work done by Agnes Ullmann in the laboratory of François Jacob and Jacques Monod , where the function of an inactive mutant β-galactosidase with deleted sequence was shown to be rescued by a fragment of β-galactosidase in which that same sequence, the α-donor peptide, is still intact. (wikipedia.org)
  • The insert of clone lambda AG18 appears to contain the full-length coding region of the processed, enzymatically active alpha-Gal A, as well as sequences coding for five amino acids of the amino-terminal propeptide, which is posttranslationally cleaved during enzyme maturation. (pnas.org)