Enzymes that catalyze the hydrolysis of N-acylhexosamine residues in N-acylhexosamides. Hexosaminidases also act on GLUCOSIDES; GALACTOSIDES; and several OLIGOSACCHARIDES.
A hexosaminidase specific for non-reducing N-acetyl-D-hexosamine residues in N-acetyl-beta-D-hexosaminides. It acts on GLUCOSIDES; GALACTOSIDES; and several OLIGOSACCHARIDES. Two specific mammalian isoenzymes of beta-N-acetylhexoaminidase are referred to as HEXOSAMINIDASE A and HEXOSAMINIDASE B. Deficiency of the type A isoenzyme causes TAY-SACHS DISEASE, while deficiency of both A and B isozymes causes SANDHOFF DISEASE. The enzyme has also been used as a tumor marker to distinguish between malignant and benign disease.
A mammalian beta-hexosaminidase isoform that is comprized of hexosaminidase beta subunits. Deficiency of hexosaminidase B due to mutations in the gene encoding the hexosaminidase beta subunit is a case of SANDHOFF DISEASE.
Conditions characterized by abnormal lipid deposition due to disturbance in lipid metabolism, such as hereditary diseases involving lysosomal enzymes required for lipid breakdown. They are classified either by the enzyme defect or by the type of lipid involved.
A beta-N-Acetylhexosaminidase that catalyzes the hydrolysis of terminal, non-reducing 2-acetamido-2-deoxy-beta-glucose residues in chitobiose and higher analogs as well as in glycoproteins. Has been used widely in structural studies on bacterial cell walls and in the study of diseases such as MUCOLIPIDOSIS and various inflammatory disorders of muscle and connective tissue.
A genus of fleas in the family Pulicidae which includes the species that serves as the primary vector of BUBONIC PLAGUE, Xenopsylla cheopis.
An autosomal recessive neurodegenerative disorder characterized by an accumulation of G(M2) GANGLIOSIDE in neurons and other tissues. It is caused by mutation in the common beta subunit of HEXOSAMINIDASE A and HEXOSAMINIDASE B. Thus this disease is also known as the O variant since both hexosaminidase A and B are missing. Clinically, it is indistinguishable from TAY-SACHS DISEASE.
The beta subunit of hexosaminidase A and hexosamininidase B. Mutations in the gene that encodes this protein are linked to SANDHOFF DISEASE and can result in loss of both hexosaminidase A activity and hexosaminidase B activity.
A tough transparent membrane surrounding the OVUM. It is penetrated by the sperm during FERTILIZATION.
A mammalian beta-hexosaminidase isoform that is a heteromeric protein comprized of both hexosaminidase alpha and hexosaminidase beta subunits. Deficiency of hexosaminidase A due to mutations in the gene encoding the hexosaminidase alpha subunit is a case of TAY-SACHS DISEASE. Deficiency of hexosaminidase A and HEXOSAMINIDASE B due to mutations in the gene encoding the hexosaminidase beta subunit is a case of SANDHOFF DISEASE.
A genus of bacteria that form a nonfragmented aerial mycelium. Many species have been identified with some being pathogenic. This genus is responsible for producing a majority of the ANTI-BACTERIAL AGENTS of practical value.
A group of related enzymes responsible for the endohydrolysis of the di-N-acetylchitobiosyl unit in high-mannose-content glycopeptides and GLYCOPROTEINS.
The facilitation of a chemical reaction by material (catalyst) that is not consumed by the reaction.
A characteristic feature of enzyme activity in relation to the kind of substrate on which the enzyme or catalytic molecule reacts.
An autosomal recessive neurodegenerative disorder characterized by the onset in infancy of an exaggerated startle response, followed by paralysis, dementia, and blindness. It is caused by mutation in the alpha subunit of the HEXOSAMINIDASE A resulting in lipid-laden ganglion cells. It is also known as the B variant (with increased HEXOSAMINIDASE B but absence of hexosaminidase A) and is strongly associated with Ashkenazic Jewish ancestry.
Proteins prepared by recombinant DNA technology.
A glycosphingolipid that accumulates due to a deficiency of hexosaminidase A or B (BETA-N-ACETYLHEXOSAMINIDASES), or GM2 activator protein, resulting in GANGLIOSIDOSES, heredity metabolic disorders that include TAY-SACHS DISEASE and SANDHOFF DISEASE.
The social process by which something or someone comes to be regarded and treated as an article of trade or commerce.
Liquid chromatographic techniques which feature high inlet pressures, high sensitivity, and high speed.
A chromatographic technique that utilizes the ability of biological molecules to bind to certain ligands specifically and reversibly. It is used in protein biochemistry. (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)
The normality of a solution with respect to HYDROGEN ions; H+. It is related to acidity measurements in most cases by pH = log 1/2[1/(H+)], where (H+) is the hydrogen ion concentration in gram equivalents per liter of solution. (McGraw-Hill Dictionary of Scientific and Technical Terms, 6th ed)
Separation technique in which the stationary phase consists of ion exchange resins. The resins contain loosely held small ions that easily exchange places with other small ions of like charge present in solutions washed over the resins.
The sum of the weight of all the atoms in a molecule.
A group of autosomal recessive lysosomal storage disorders marked by the accumulation of GANGLIOSIDES. They are caused by impaired enzymes or defective cofactors required for normal ganglioside degradation in the LYSOSOMES. Gangliosidoses are classified by the specific ganglioside accumulated in the defective degradation pathway.
The alpha subunit of hexosaminidase A. Mutations in the gene that encodes this protein can result in loss of hexosaminidase A activity and are linked to TAY-SACHS DISEASE.
Clinical or subclinical disturbances of cortical function due to a sudden, abnormal, excessive, and disorganized discharge of brain cells. Clinical manifestations include abnormal motor, sensory and psychic phenomena. Recurrent seizures are usually referred to as EPILEPSY or "seizure disorder."
A disorder characterized by the onset of myoclonus in adolescence, a marked increase in the incidence of absence seizures (see EPILEPSY, ABSENCE), and generalized major motor seizures (see EPILEPSY, TONIC-CLONIC). The myoclonic episodes tend to occur shortly after awakening. Seizures tend to be aggravated by sleep deprivation and alcohol consumption. Hereditary and sporadic forms have been identified. (From Adams et al., Principles of Neurology, 6th ed, p323)
Recording of electric currents developed in the brain by means of electrodes applied to the scalp, to the surface of the brain, or placed within the substance of the brain.
A childhood seizure disorder characterized by rhythmic electrical brain discharges of generalized onset. Clinical features include a sudden cessation of ongoing activity usually without loss of postural tone. Rhythmic blinking of the eyelids or lip smacking frequently accompanies the SEIZURES. The usual duration is 5-10 seconds, and multiple episodes may occur daily. Juvenile absence epilepsy is characterized by the juvenile onset of absence seizures and an increased incidence of myoclonus and tonic-clonic seizures. (Menkes, Textbook of Child Neurology, 5th ed, p736)
A linear polysaccharide of beta-1->4 linked units of ACETYLGLUCOSAMINE. It is the second most abundant biopolymer on earth, found especially in INSECTS and FUNGI. When deacetylated it is called CHITOSAN.
A hexosiminidase that specifically hydrolyzes terminal non-reducing N-acetyl-D-galactosamine residues in N-acetyl-beta-D-galactosaminides.
A genus of silkworm MOTHS in the family Bombycidae of the order LEPIDOPTERA. The family contains a single species, Bombyx mori from the Greek for silkworm + mulberry tree (on which it feeds). A native of Asia, it is sometimes reared in this country. It has long been raised for its SILK and after centuries of domestication it probably does not exist in nature. It is used extensively in experimental GENETICS. (From Borror et al., An Introduction to the Study of Insects, 4th ed, p519)
An enzyme that converts UDP glucosamine into chitin and UDP. EC 2.4.1.16.
Insects of the suborder Heterocera of the order LEPIDOPTERA.

Strong induction of members of the chitinase family of proteins in atherosclerosis: chitotriosidase and human cartilage gp-39 expressed in lesion macrophages. (1/897)

Atherosclerosis is initiated by the infiltration of monocytes into the subendothelial space of the vessel wall and subsequent lipid accumulation of the activated macrophages. The molecular mechanisms involved in the anomalous behavior of macrophages in atherogenesis have only partially been disclosed. Chitotriosidase and human cartilage gp-39 (HC gp-39) are members of the chitinase family of proteins and are expressed in lipid-laden macrophages accumulated in various organs during Gaucher disease. In addition, as shown in this study, chitotriosidase and HC gp-39 can be induced with distinct kinetics in cultured macrophages. We investigated the expression of these chitinase-like genes in the human atherosclerotic vessel wall by in situ hybridizations on atherosclerotic specimens derived from femoral artery (4 specimens), aorta (4 specimens), iliac artery (3 specimens), carotid artery (4 specimens), and coronary artery (1 specimen), as well as 5 specimens derived from apparently normal vascular tissue. We show for the first time that chitotriosidase and HC gp-39 expression was strongly upregulated in distinct subsets of macrophages in the atherosclerotic plaque. The expression patterns of chitotriosidase and HC gp-39 were compared and shown to be different from the patterns observed for the extracellular matrix protein osteopontin and the macrophage marker tartrate-resistant acid phosphatase. Our data emphasize the remarkable phenotypic variation among macrophages present in the atherosclerotic lesion. Furthermore, chitotriosidase enzyme activity was shown to be elevated up to 55-fold in extracts of atherosclerotic tissue. Although a function for chitotriosidase and HC gp-39 has not been identified, we hypothesize a role in cell migration and tissue remodeling during atherogenesis.  (+info)

Purification and chemical characterization of human hexosaminidases A and B. (2/897)

N-Acetyl-beta-hexosaminidases A and B were purified to homogeneity from human placenta. In the initial step of purification, the enzymes were adsorbed on concanavalin A-Sepharose 4B and eluted from the column with alpha-methyl D-mannosides. Subsequent purification steps included DEAE-cellulose column chromatography, QAE-Sephadex [diethyl-(2-hydroxypropyl)aminoethyl-Sephadex] column chromatography, Sephadex G-200 gel filtration and preparative disc polyacrylamide-gel electrophoresis, followed by another QAE-Sephadex chromatography for the hexosaminidase A preparation, and DEAE-cellulose column chromatography, calcium phosphate gel chromatography, Sephadex G-200 gel filtration, QAE-Sephadex chromatography and CM-cellulose chromatography for the hexosaminidase B preparation. The purified preparations, particularly hexosaminidase A, had significantly higher specific enzyme activities than previously reported. The preparations moved on polyacrylamide-gel electrophoresis as single protein bands, which also stained for enzyme activity. Sedimentation-equilibrium centrifugation indicated homogenous dispersion of the enzymes, and the molecular weight was estimated as about 110000 for both enzymes. Complete amino acid and carbohydrate compositions of the two isoenzymes were determined, and, in contrast with previous suggestions, no sialic acid was found in the enzymes.  (+info)

The subunits of human hexosaminidase A. (3/897)

Previous studies of the subunit structure of hexosaminidase gave ambiguous results, but suggested that the enzyme was composed of six equally sized subunits. Dissociation of hexosaminidase A with p-chloromercuribenzoate produces an alkylated fragment with mol.wt. approx. 50000, which is converted into hexosaminidase S by treatment with dithiothreitol. Treatment of native hexosaminidase A with sodium dodecylsulphate results in the formation of a large and a small fragment. However, although the native enzyme has a sedimentation coefficient of 5.8S, dissociation by S-carboxymethylation and maleic anhydride treatment results in subunits exhibiting a single schlieren boundary on analytical ultracentrifugation with a sedimentation coefficient of 2.18S. These results indicate that the enzyme is composed of four subunits, each with molwt. approx. 25000-27000. The mol.wt. of the native enzymes is calculated to be approx. 110000. Our data are consistent with the subunit structures of hexosaminidases A, B and S as being alpha2beta2, beta4 and alpha4 respectively.  (+info)

Biochemical characterization of CD1d expression in the absence of beta2-microglobulin. (4/897)

CD1d is a major histocompatibility complex class I-like molecule that exhibits a distinct antigen processing pathway that functions in the presentation of hydrophobic antigens to T cells. CD1d has been previously shown to be expressed on the cell surface of human intestinal epithelial cell lines in vivo and a transfected cell line in vitro independently of beta2-microglobulin (beta2m). To define the relationship between CD1d and beta2m and characterize the biochemical structure of CD1d in the absence of beta2m, we have used a newly generated series of CD1d transfectants and CD1d-specific antibodies. These studies show that in the absence of beta2m, CD1d is expressed on the cell surface as a 45-kDa glycoprotein that is sensitive to endoglycosidase-H and is reduced to 37-kDa after N-glycanase digestion. In contrast, in the presence of beta2m, CD1d is expressed on the cell surface as a 48-kDa endoglycosidase-H-resistant glycoprotein. Pulse-chase metabolic labeling studies demonstrate that acquisition of endoglycosidase-H resistance of CD1d is observed in the presence of beta2m but not in the absence of beta2m even after a 24-h chase period. Thus, CD1d is able to be transported to the cell surface independently of beta2m; however, in the absence of beta2m, the glycosylation pattern of CD1d is altered and consistent with an immature glycoprotein.  (+info)

Intracellular formation and processing of the heterotrimeric gH-gL-gO (gCIII) glycoprotein envelope complex of human cytomegalovirus. (5/897)

The human cytomegalovirus (HCMV) gCIII complex contains glycoprotein H (gH; gpUL75), glycoprotein L (gL; gpUL115), and glycoprotein O (gO; gpUL74). To examine how gH, gL, and gO interact within HCMV-infected cells to assemble the tripartite complex, pulse-chase experiments were performed. These analyses demonstrated that gH and gL associate by the end of the pulse period to form a disulfide dependent gH-gL complex. Subsequently, the gH-gL complex interacts with a 100-kDa precursor form of gO to form a 220-kDa precursor of the mature gH-gL-gO complex that contains a 125-kDa form of gO. The 220-kDa precursor complex (pgCIII) was sensitive to treatment with endoglycosidase H (endo H), while the mature gCIII complex was essentially resistant to digestion with this enzyme, suggesting that formation of pgCIII complex occurs in the endoplasmic reticulum (ER) and is processed to mature gH-gL-gO (gCIII) in a post-ER compartment. While the N-linked glycans on the 100-kDa form of gO were modified to endo H-resistant states as the 125-kDa gO formed, additional posttranslational modifications were detected on gO. These processing alterations were non-N-linked oligosaccharide modifications that could not be accounted for by phosphorylation or by O-glycosylation of the type sensitive to O-glycanase. Of gH, gL, gO, and the various complexes that they form, only the mature form of the complex was detectable at the infected cell membrane, as judged by surface biotinylation studies.  (+info)

N-Benzoyl-L-tyrosyl-p-aminobenzoic acid hydrolase beta (human meprinbeta). A 13-amino-acid sequence is required for proteolyticprocessing and subsequent secretion. (6/897)

N-Benzoyl-L-tyrosyl-p-aminobenzoic acid hydrolase or human meprin (PPH) is a brush-border membrane enzyme of small intestinal epithelial cells. It is a type I integral membrane protein composed of two disulphide-bridged subunits (alpha and beta). PPH and its homologous counterparts in rodents belong to the astacin family of zinc-metalloendopeptidases. Although the amino-acid sequence of the beta subunits is 80-90% identical in these three species, processing is different. Expression of PPHbeta in simian virus 40-transformed African green monkey kidney cells (COS-1) and Madin Darby canine kidney (MDCK) cells results in its cell surface localization and secretion, whereas mouse meprinbeta is only found at the plasma membrane. To investigate proteolytic processing of PPHbeta and to identify the cleavage site, different C-terminal domains of wild-type PPHbeta were exchanged with the homologous domains of mouse meprinbeta. We identified a 13-amino-acid sequence (QIQLTPAPSVQDL) necessary for cleavage and subsequent secretion of PPHbeta. Using brefeldin A, the site of processing was identified as being after passage through the Golgi compartment. Proteolytic processing of PPHbeta thus provides a means for secretion of alphabeta heterodimers.  (+info)

Trypanosoma cruzi calreticulin is a lectin that binds monoglucosylated oligosaccharides but not protein moieties of glycoproteins. (7/897)

Trypanosoma cruzi is a protozoan parasite that belongs to an early branch in evolution. Although it lacks several features of the pathway of protein N-glycosylation and oligosaccharide processing present in the endoplasmic reticulum of higher eukaryotes, it displays UDP-Glc:glycoprotein glucosyltransferase and glucosidase II activities. It is herewith reported that this protozoan also expresses a calreticulin-like molecule, the third component of the quality control of glycoprotein folding. No calnexin-encoding gene was detected. Recombinant T. cruzi calreticulin specifically recognized free monoglucosylated high-mannose-type oligosaccharides. Addition of anti-calreticulin serum to extracts obtained from cells pulse-chased with [35S]Met plus [35S]Cys immunoprecipitated two proteins that were identified as calreticulin and the lysosomal proteinase cruzipain (a major soluble glycoprotein). The latter but not the former protein disappeared from immunoprecipitates upon chasing cells. Contrary to what happens in mammalian cells, addition of the glucosidase II inhibitor 1-deoxynojirimycin promoted calreticulin-cruzipain interaction. This result is consistent with the known pathway of protein N-glycosylation and oligosaccharide processing occurring in T. cruzi. A treatment of the calreticulin-cruzipain complexes with endo-beta-N-acetylglucosaminidase H either before or after addition of anti-calreticulin serum completely disrupted calreticulin-cruzipain interaction. In addition, mature monoglucosylated but not unglucosylated cruzipain isolated from lysosomes was found to interact with recombinant calreticulin. It was concluded that the quality control of glycoprotein folding appeared early in evolution, and that T. cruzi calreticulin binds monoglucosylated oligosaccharides but not the protein moiety of cruzipain. Furthermore, evidence is presented indicating that glucosyltransferase glucosylated cruzipain at its last folding stages.  (+info)

Use of inhibitors to characterize intermediates in the processing of N-glycans synthesized by insect cells: a metabolic study with Sf9 cell line. (8/897)

The most frequent type of N-glycan synthesized by lepidopteran Sf9 cells appears to be fucosylated Man3GlcNAc2,and this has been a limitation for a large scale production and utilization of therapeutic glycoproteins in cultured insect cells. The current knowledge of the protein glycosylation pathway derived from structural studies on recombinant glyco-proteins expressed by using baculovirus vectors. In this work we provide more direct evidence for the sequential events occurring in the processing of endogenous N-glycoproteins of noninfected Sf9 cells. By metabolic labeling with radioactive mannose, we characterized the glycan structures which accumulated in the presence of processing inhibitors (castanospermine and swainsonine) and in the presence of an intracellular trafficking inhibitor (monensin). We thus demonstrated that from the glycan precursor Glc3Man9GlcNAc2 to GlcNAcMan5(Fuc)GlcNAc2 intermediate, the processing pathway in Sf9 cells paralleled the one demonstrated in mammalian cells. By using monensin, we demonstrated the formation of Man3(Fuc)GlcNAc2 from GlcNAcMan3(Fuc)GlcNAc2, a reaction which has not been described in mammalian cells. Our results support the idea that the hexosaminidase activity is of physiological relevance to the glycosylation pathway and is Golgi located.  (+info)

Microglia is thought to be important in Alzheimers disease. Therefore, our aim was to investigate the usefulness of the microglial marker chitotriosidase in clinical trials. Chitotriosidase was analyzed in cerebrospinal fluid from Alzheimers disease patients on acetylcholine esterase inhibitors (AChEI) and in cerebrospinal fluid from multiple sclerosis patients before and after natalizumab treatment. Chitotriosidase activity was extremely stable during treatment with the non-inflammatory drug AChEI. However, the immunomodulatory treatment with natalizumab led to lower chitotriosidase activity. Thus, chitotriosidase may be useful in clinical trials where microglia is targeted or as a safety biomarker in other trials where the brain is a bystander organ.. ...
Active site of hexosaminidases with dockedpNP-GlcNAc-sulfate 6. Active site of hexosaminidases with docked pNP-GlcNAc-sulfate 6 after molecular dynamics simulat
Chit - Definition of Chit and synonyms of Chit are presented by online Websters Dictionary. Includes dictionary browser, morphological search by meaning of Chit, thesaurus, related words, and dictionary browser. Provides Chit usage examples
ACHAMMA KOSHY, DONALD ROBINSON, JOHN L. STIRLING; An Attempt to Purify N-Acetyl-β-hexosaminidases from Crude Extracts of Human Liver by Affinty Chromatography. Biochem Soc Trans 1 April 1975; 3 (2): 244-246. doi: https://doi.org/10.1042/bst0030244. Download citation file:. ...
Portia H. Fernandez-Marcelo. National Telehealth Center, National Institutes of Health, University of the Philippines Manila. The CHITS (Community Health Information and Tracking System), the first electronic medical record system in the Philippines that is used widely, has persevered through time and slowly extended its geographic footprint, even without a national policy. This study describes the process of CHITS development, its enabling factors and challenges affecting its adoption, and its continuing use and expansion through eight years of implementation (2004 to 2012) using the HOT-fit model. This paper used a case study approach. CHITS was developed through a collaborative and participative user-centric strategies. Increased efficiency, improved data quality, streamlined records management and improved morale among government health workers are benefits attributed to CHITS. Its longevity and expansion through peer and local policy adoption speaks of an eHealth technology built for and by ...
O:13:\PanistOpenUrl\:36:{s:10:\\u0000*\u0000openUrl\;N;s:6:\\u0000*\u0000idc\;N;s:6:\\u0000*\u0000fmt\;s:7:\journal\;s:6:\\u0000*\u0000doi\;s:17:\10.1159\/000011956\;s:6:\\u0000*\u0000pii\;s:0:\\;s:7:\\u0000*\u0000pmid\;s:0:\\;s:9:\\u0000*\u0000atitle\;s:82:\N-acetyl-\u03b2-hexosaminidase activity and isoenzymes in human gastric adenocarcinoma\;s:9:\\u0000*\u0000jtitle\;s:8:\Oncology\;s:9:\\u0000*\u0000stitle\;s:8:\Oncology\;s:7:\\u0000*\u0000date\;s:4:\1999\;s:9:\\u0000*\u0000volume\;s:2:\56\;s:8:\\u0000*\u0000issue\;s:1:\2\;s:8:\\u0000*\u0000spage\;s:3:\142\;s:8:\\u0000*\u0000epage\;s:3:\154\;s:8:\\u0000*\u0000pages\;s:0:\\;s:7:\\u0000*\u0000issn\;s:9:\0030-2414\;s:8:\\u0000*\u0000eissn\;s:0:\\;s:9:\\u0000*\u0000aulast\;s:10:\GIL-MARTIN\;s:10:\\u0000*\u0000aufirst\;s:1:\E\;s:9:\\u0000*\u0000auinit\;s:0:\\;s:10:\\u0000*\u0000auinitm\;s:0:\\;s:5:\\u0000*\u0000au\;a:3:{i:0;s:24:\RODRIGUEZ-BERROCAL, F. ...
Learn about InsaneScouter - Firemn Chit Course at InsaneScouter.org! Firemn Chit Course Objective Instruct students on proper use and safety of heat sources ...
购买Abcam重组人CHIT1蛋白(ab116982),经WB,ELISA,SDS-PAGE验证。蛋白片段。提供28,000多种信号蛋白、细胞因子、趋化因子、生长因子等产品。
Page 11 of 12 - How many share same beliefs with SO? - posted in Chit Chat - Public: My partner encourages and supports my craft. We share many of the same beliefs, and when we dont we respect our differences. She is more agnostic while I am atheist when it comes to religion, which has never played a big part of our relationship. We have been together for 8 years now, so we must be doing something right.
We seek the download Chit helpful ferry in this organization: as an helpful justice that is both the default of safer management and the things and omnis that will be its error and book by community. Although there have a information of mathematical, industrial, medical, and Chaotic issues to the free theory of Russian repertoire bodies by system( Matus et al. 2007), repackaging these insights not will also exist random to share the Liberalism life. Bairnsdale these courses will show attention and important consultants to be the world of tools in the schools health.
Enforcement Directorate (ED) has attached Rs 300 crore assets of Rose Valley group in its ongoing probe against the chit fund company for money laundering
Chitotriosidase is secreted by activated human macrophages and is markedly elevated in plasma of Gaucher disease patients. The expression of chitotriosidase occurs only at a late stage of differentiation of monocytes to activated macrophages in culture. Human macrophages can synthesize a functional chitotriosidase, a highly conserved enzyme with a strongly regulated expression. This enzyme may play a role in the degradation of chitin-containing pathogens. Several alternatively spliced transcript variants have been described for this gene. [provided by RefSeq, Jan 2012 ...
FUNCTION: [Summary is not available for the mouse gene. This summary is for the human ortholog.] Chitotriosidase is secreted by activated human macrophages and is markedly elevated in plasma of Gaucher disease patients. The expression of chitotriosidase occurs only at a late stage of differentiation of monocytes to activated macrophages in culture. Human macrophages can synthesize a functional chitotriosidase, a highly conserved enzyme with a strongly regulated expression. This enzyme may play a role in the degradation of chitin-containing pathogens. Several alternatively spliced transcript variants have been described for this gene. [provided by RefSeq, Jan 2012 ...
Part of our trip was 3 nights on the Disney Wonder Cruise Ship, I did a review of our cute little Stateroom with Verandah HERE. If you are considering a Disney Cruise in the future, its nice to see what your room will be like.. ...
Hows things going ??,, they are going here,, mud,, lots of it,, seems we start to dry out,, then we get more rain..:disturbed: My sister was once again in the hospital,, they put a pacemaker in,, she is handling the best she can,,she went home today,,,, anyone hear of a new MS drug approved ? My SIL told me it was on the news,, I havent heard.. better go,, later,,
၀မ္းသြားခ်င္တာကိုေအာင့္ထားျခင္းက နာတာရွည္ ၀မ္းခ်ဳပ္ေရာဂါကိုျဖစ္ေစႏိုင္ျပီး ခြဲစိတ္မွဳ ခံယူရတဲ့အထိျဖစ္ႏုိင္
This ones anew wrinkle. Got a laptop infected with twunk_32.exe and wscsvc32.exe, both of which seem pretty new. Anyway, this laptop has two partitions, a 76gb fat32 c: drive and a 75gb fat32 d: drive (yes, fat32!). Windows wont boot and bluescreens instead. Both partitions are mountable from a PE disk, and the data is there. So Im running a backup right now and look to convert the c: drive back to ntfs. Never seen this chit though. Anyone else?
So since its raining outside and my saddle rails have been shot to chit, Ive been doing some reading. There have been a number of such threads in
Iam currently living at home and my current situation has lead me to look for a place to move into. I work full time as an automotive master technician normal hours of 8 - 4:30. I generally tend to stay home after work during the week and go out on the weekends to catch up with friends. Very flexible and super easy going, not a crazy neat freak but I like to keep my place clean and tidy. Im respectful of others and always take into consideration of the things around me. Like to have a chit chat and have a meal together but dont mind also doing my own thing. Massive car guy so secure parking is a must for me. Thanks ...
T04128 (actw,agd,agv,alg,amar,bez,bgz,bman,brf,chit,crs,day,dic,djj,dou,ebv,eex,elk,fmi,fnf,gmr,gti,hhk,hhz,hmi,kuy,lgt,lne,lyj,lyk,marj,metf,mff,mgk,mmaa,mmak,mmao,ncu,nfv,ngf,nik,niw,noi,nst,ock,oct,oli,otr,pacr,paut,peh,peu,prag,prd,psyh,pxv,ree,rfs,rhf,rhg,rid,rmuc,rul,salz,seqi,serw,smus,snn,sper,sphz,spik,thet : calculation not yet completed ...
Daniel Hunt, André Knuchel-Takano, Abbygail Jaccard, Arti Bhimjiyani, Lise Retat, Chit Selvarajah, Katrina Brown, Laura L Webber, Martin Brown ...
VERA Files trustee and journalism professor Lourdes Chit Estella-Simbulan, a journalist of three decades, was known to the public by her byline Chit Estella. She was killed on the evening of May 13, 2011 when a Universal Guiding Star bus rammed the taxi she was in near the Ayala Technohub on Commonwealth Avenue, Quezon City. This page provides deeper analysis of the causes and impacts of road crashes -- a leading cause of death worldwide. This project is produced under the Bloomberg Initiative Global Road Safety Media Fellowship implemented by the World Health Organization, Department of Transportation and VERA Files.
Opens the Highlight Feature Bar and highlights feature annotations from the FEATURES table of the record. The Highlight Feature Bar can be used to navigate to and highlight other features and provides links to display the highlighted region separately. Links in the FEATURES table will also highlight the corresponding region of the sequence. More... ...
Buy NAG elisa kit, Canine N-Acetyl-beta-D-Glucosaminidase ELISA Kit-AAS02154.1 (MBS005370) product datasheet at MyBioSource, ELISA Kits
Hello and to the Jaded Ladies site! We are a group of ladies that come here for support and encouragement on our weight loss struggles, and we also
Hello and Welcome to the Jaded Ladies thread to anyone reading this! We are a very supportive and caring bunch of ladies who share our journey with
Hi Everyone Hope you all have had a lovely weekend. I was very lucky last month and v kindly received the Award Winning *Nourishing Cleanser (Full Size 100ml RRP £46) from Jane Scrivner, along with other samples from her range. Now that I have had to chance to try it properly wanted to let you know my thoughts.…
I have a circuit (see pic) that includes the primary winding of a 1:20 self wound transformer. Without some form of additional resistance this circuit will draw too much current from the power supply and damage the FET Im using so I plan to put a rheostat inline with the primary winding to contr...
All the equipment filled up a room, and didnt have any computing power as we know it today instead it did addition by repeated increments of the same command over and over again rather than having any multiplying hardware.. I remember 8 floppys , then 5.25 and I even owned a dual 5.25 IEEE drive, then my first Hard drive was a 30 mb , it took a couple years to fill it, and , used to operate a PC bulletin Board back in the early 80s. The worlds first gigabyte-capacity disk drive, the IBM 3380, introduced in 1980, was the size of a refrigerator, weighed 550 pounds (about 250 kg), and had a price tag of $40,000. (I didnt own one), today I have a 300 gig hdd its 1 thick and fits in a shirt pocket I paid $110.USD for it.. So no need to feel old, Yes we have seen a lot and dont forget you had to change the channels on the TV with pliers because the knob was striped or broken and even had to get up to do it. These are things that give us experience and the knowledge to help others. without us, ...
Ha, You took my spot. And I actually mean that. I was in troop 10, set to fly out Sunday and yesterday I had to have surgery to have my appendix removed. I was told that I need my doc to sign off, then see the RCMPs doctor to sign off and I will be given another troop ...
Howdy,, hows the world treating you? Going good here,,no big complaints, we have been doing fall clean up,,the garden is stripped and all we have are a few tomatoes and peppers,, oh yes butternut squash,, we ended up with over 75 of them , trying to figure out what to do with all of them ! Grandkids are here, riding horses, and doing the youth deer hunting with grandpa,,so far the deer is winning,, Weather is finally cooling down ,, oh on a cuteness note,, I tried to get a picture
N-acetyl β-glucosaminidase (EC 3.2.1.52); hyaluronidase (EC 3.2.1.35); [protein]-3-O-(GlcNAc)-L-Ser/Thr β-N-acetylglucosaminidase (EC 3.2.1.169 ...
In March 2009, the then Home Secretary requested advice from the Advisory Council on the Misuse of Drugs (ACMD) on so called legal highs. …
Werries, E., Wollek, E., Gottschalk, A. and Buddecke, E. (1969). „Separation of N-acetyl-α-glucosaminidase and N-acetyl-α-galactosaminidase from ox spleen. Cleavage of the O-glycosidic linkage between carbohydrate and polypeptide in ovine and bovine submaxillary glycoprotein by N-acetyl-α-galactosaminidase. Eur. J. Biochem. 10: 445-449. PMID 5348072 ...
β-galactosidase (EC 3.2.1.23); exo-β-glucosaminidase (EC 3.2.1.165); exo-β-1,4-galactanase (EC 3.2.1.-); β-1,3-galactosidase (EC 3.2.1.- ...
This is from a post on Chit Chat. I let the bread machine mix a double batch of dough and do a first rising. I took the dough out and kneaded for
Page 2 of 3 - O- Blood - posted in Chit Chat - Public: Do you know what? I have no idea what my blood type is!! Is that bad???? Talking about eye colour, my eyes were blue till i was 15 and now they are green. Maybe they change with age. Not sure if they change when im angry though, never checked myself out in the mirror when im going into one lol!.
Coyotes out at Sycamore Canyon and Goodan Ranch are eating some yellow fruit that is creating some nasty chit on the trails. I almost got queezy
Mae cetamin hefyd yn cael ei ddefnyddio fel cyffur hamdden anghyfreithiol[6]. Yn y Deyrnas Unedig, fei labelwyd yn gyffur Dosbarth C ar 1 Ionawr 2006. Ar 10 Rhagfyr 2013, argymhellodd Cyngor Ymgynghorol y DU ar Gamddefnyddio Cyffuriau (ACMD) ir llywodraeth ail-ddosbarthu cetamin i fod yn gyffur Dosbarth B, ac ar 12 Chwefror 2014 cyhoeddodd y Swyddfa Gartref y byddent yn dilyn yr argymhelliad yng ngoleunir dystiolaeth o ddamweiniau cronig syn gysylltiedig â defnyddio cetaminau, gan gynnwys difrod cronig ir bledren a rhannau eraill or llwybr wrinol Fel cyffur hamddenol maen cael ei hadnabod wrth yr enwau y grintach werdd, rockmesc, K, Citi, Kallie Ziltz, Cet, Special K, K2, Fitamin K, Super K, Piws, Skittles, olew mêl ac enwau eraill. Wedi ei gymysgu a chocên maen cael ei alwn CK neu Calvin Klein. ...
Schindler Disease Type 1 (Alpha-N-Acetylgalactosaminidase Deficiency Type 1): Read more about Symptoms, Diagnosis, Treatment, Complications, Causes and Prognosis.
What is the definition of HEXOSAMINIDASE? What is the meaning of HEXOSAMINIDASE? How do you use HEXOSAMINIDASE in a sentence? What are synonyms for HEXOSAMINIDASE?
Mono- and Stereopictres of 5.0 Angstrom coordination sphere of Sodium atom in PDB 3ecq: Endo-Alpha-N-Acetylgalactosaminidase From Streptococcus Pneumoniae: Semet Structure
This weeks Chit Chat Across the Pond is a fantastic blend of technology, physiology and ingenuity to solve a very common problem. Were joined by Terry Austin, a professor of Anatomy & Physiology and microbiology at Temple College in the Texas Hill Country. He turned his research background in neuroscience and his love of technology toward a solution for a very personal problem to be solved.. Terry has some hearing loss, but not bad enough (in his estimation) to warrant hearing aids. He went on a hunt for an app to help. He found Hearing Aid Plus from hearingaidproapp.com for iOS.. I dont want to steal his entire story, but in this episode he tells us how his knowledge of physiology combined with the mad skills of developer Gábor Száanto may have come up with a breakthrough solution for those with non-cochlear hearing loss.. Terry tells the whole story on his blog at intelligentelectrons.com/…. Also referenced in the recording: Trekz Titanium bone-conducting headphones: ...
Studies are reported on Alpha-Galactosidase and Conversion of Group B Erythrocytes and (2) Isolation and Characterization of Alpha-N-Acetylgalactosaminidase.*Enzymes
Hexosaminidase A and B answers are found in the Daviss Lab & Diagnostic Tests powered by Unbound Medicine. Available for iPhone, iPad, Android, and Web.
Sujiaonori, a river alga growing in the Kochi prefecture, Japan, contains several bioactive compounds such as sulfated polysaccharides (ulvans), ω-3 fatty acids, and vitamins. Dietary intake of this alga-based supplement has been reported to increase circulatory adiponectin, a salutary hormone that is reported to be associated with healthy longevity and prevents a number of cardiovascular and metabolic disorders. This report highlights the anti-allergic and skin health enhancing effects of Sujiaonori-derived ulvan (Tosalvan) and supplement, respectively. RBL-2H3 cell line was used to investigate the anti-allergic effect of algal SP through the evaluation of β-hexosaminidase activity. Algal sulfated polysaccharides or SP (Tosalvan, Yoshino SP) were extracted from powders of dried alga samples provided by local food manufacturers. Report on the effect of daily dietary intake of Sujiaonori-based supplement on skin health is part of a four-week clinical investigation that, in comparison with a supplement
Thank you for your interest in spreading the word about Biochemical Society Transactions.. NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.. ...
Chitosan-degrading fungal strain, Penicillium sp. IB-37-2A, produced mainly extracellular chitosanolytic enzymes under submerged agitating cultivation in p
Where do old airplanes go? The lucky ones go to the Air Mobility Command Museum-the only museum dedicated to airlift and air refueling history!
I was really entertained and more by reading a few pages. I am totally pleased finding that you have a beagle hound. I love beagles as I raised and field trialed them and hunted them for 20+ yrs. Give your beagle a chance and it will give you mountain music, its called, from trailing open-mouthed game like rabbits. Many have a nice chop mouth and many have a beautiful bawl mouth when trailing. Generally Ive found a beag. that bawls in the kennel will chop on trail and vice versa. Yours is a very pretty, well marked beagle with light colored head and black blanket back, practically always tri-colored. Im 68 years old/young now and am converting my 100ft by 50ft garden into a Back to Eden garden in an effort to conserve water. We have been in a severe drought the past 2 years and water is now very expensive, too expensive to water everything as it needs it in the 100 degree days of summer like weve had for last 2 years. Looking for ideas to shade my garden with either pole beans or grape ...
Well the sad fact is as long as one will give in the inmates will pull their crap. Flooding cells being loud and all that crazy crap. I myself go with the saying I do not negotiate with terrorist. If they have rights to it such as food, safety, and basic health care then as I read my post orders it is what I am paid to provide. The extra stuff like fluffing pillows and bending over backwards to be a personal assistant well not happening.. Had an inmate who needed to talk to the head of the dog program to get vet visits arranged I sadly was kind enough to arrange the meeting over the phone. Well after about 5 minutes of him tying up my phone line I was paged to call the captains office I told him to stop chit chatting and get off the phone. He was upset about this and even the program director had to call me back and explain they were not chit chatting. I explained I do not have tolerance for inmates who have an undeserved sense of entitlement and figured that talking about how a certain dogs ...
[QUOTE=tofeeees]@Sonali: all moms hate what their daughters chose to wear LOLL! and yup make up is sucha big issue in my life! ub toh i do it myself cuz i work but pehlay toh gosh![/QUOTE]i agree my mums never happy either- sighmy dads sweet tho- he loves u if u wear anything blue so hes ... | Page: 33 | 1635249 | Rang Badalti Odhani Forum
Latest News, World , Asia, ASEAN, Singapore,Phillipines, Malaysia , Indonesia, Thailand, Vietnam, Taiwan, Hong Kong,China and India News Headlines. Latest on Sports, Politics, Science and Technology and other things around the globe.
General Information: This strain was isolated from the Baltic Sea. A psychrophilic bacterium. This genus includes species that inhabit a wide range of environments and are capable of utilizing a wide variety of electron acceptors during anaerobic respiration including some insoluble metal oxides while using very few carbon sources such as lactate or acetate. This group of organisms have been studied extensively for their electron transport systems.This species is differentiated from other Shewanella spp. based on its ability to grow at 4 degrees C but not at 37, production of N-acetyl-beta-glucosaminidase, lack of chymotrypsin, and ability to use a variety of complex carbon compounds as carbon and energy sources. ...
HEXB antibody (hexosaminidase B (beta polypeptide)) for ICC/IF, IHC-P, WB. Anti-HEXB pAb (GTX55224) is tested in Human samples. 100% Ab-Assurance.
The researchers, led by Dr Cheok Chit Fang and Prof David Lane, the co-discoverer of the p53 gene in 1979, achieved this by exploiting one of the key functions of p53 the control of the cell cycle. Activating p53 halts the cell cycle and prevents endoreduplication, a process by which a cell accumulates excess genetic material by duplicating its existing genetic material without actually dividing. If endoreduplication happens in human cells, they die. Deliberately inducing endoreduplication in cancer cells through chemical means has been explored as a means of killing off cancer cells. However, as the drugs used are not highly specific to cancer cells, many normal cells are also killed in the process. Fortunately, in many cancers, the cancer cells lack working copies of p53. By using a drug that activates p53 in healthy cells and temporarily induces the cells to stop the production of genetic material, endoreduplication is prevented. Cancer cells which lack working copies of p53 are thus left ...
Pathogenic bacteria are proliferating due to chemical chit chat called quorum sensing. Researchers are scrambling for a way to silence them.
Q: Can you give us some comments on (Dr Chees) comments as well, regarding the managing agents for town councils, and investments made?. PM Lee: All that is just by the by. The key thing is who has the character, who has the track record, who can you trust. And the PAP town councils will manage it to the best of our ability, we have our reputation at stake.. I dont say we never make mistakes, but when we do make mistakes, we put them right, and I think overall our track record of managing town councils is good. And if you ask the residents here, I think the residents are happy with the work the town councils have done, and which the MPs have done, working with them to improve the neighbourhood.. I was last here in 2009, after Ong Chit Chung passed away I came and took a look around, just to make sure everything is alright, and to show our support for people. And between 2009 and now, there have been improvements made to this place. We have the amphitheatre and multi-purpose hall that was not ...
Snapchat Hacked January 2017. Get some return for the time that theyre spending in that neighborhood. How do you balance between making sure that youre giving a thing back to the group, and likewise surroundings your self up in a way that individuals want to buy from you? Are you placing out some call to action at the tip for them to buy? They say because that could be a great deal of a hard sale, like a hard pitch. How do you stability between growing value and never pissing off every person on Reddit but in addition making sure youre still getting some return on the investment that you simplyre inserting into the group. Kevin: We love to put up a chit code, like a bargain that includes our posts. It is lots about giving back. We get successful information there, and I think adding value to the community is essential but in addition giving them the chance to buy what youre promoting and get real world advice is large. We made a test post, well I did, on r/todayilearned, and I used one of ...
NOT letting go on July 13, 1998…. Dear Breakup Girl,. I know that Im on your Breakup List and I wholeheartedly agree - and the deed was carried out a couple of weeks ago. We agreed to be friends and in an effort to talk (we estimated an hour) we ended up spending most of the day (and night) together - good idea…at the time. So in parting, we really let loose on the feelings while in an hour long embrace - I cried, he choked up - the whole 9 yards.. Well, Id closed the chapter - not to look back ANYTIME SOON, when who appears at my office(almost missing me) we ran into each other as Im getting off the elevator and hes getting on. He was with a friend and I was with his sis (who I work with - tough to forget with her around). He says that he misses me and was going to call me the night before, etc. We all chit chat and sis and I go in the office. So on my desk is a note saying that he wished I was here, that he missed our talks, he was looking forward to seeing me, ending it with ...
Then theres the people who obviously dont have dogs of their own but think your dog is solely for their (or their childrens) entertainment. Seriously people? What is it about our look that SCREAMS stop my dog while were BOTH in a full on run, his leash clipped to my waist then watch as my head snaps back hitting my spine as I pinball it back to my dog who is now eating gold fish crackers off your kids stroller tray??? Im glad you think thats so cute because sure as shit it feels like I cracked a vertebrae in my neck thanks to you. Dont misunderstand, I LOVE stopping to chit chat with people when Wrigs and I are out for a leisurely stroll. But when Im running full force, staring straight ahead, ear buds and all, I really dont think its a good idea to jump in front of my dog and ask if your kids can pet him. And so I stand there, out of breath, fiddling with my i-pod strapped to my arm while you start talking nonsense but guess what? I cant hear a word you are saying so I just shout, ...
No password needed. This meeting has changed format and will be based around A Quiet Peace reading and will be 1 hour in duration.. Chit system.. ...
For all routine treatments and repeat prescriptions, pupils are encouraged to come to the Medical Centre at surgery times. Emergencies and sudden illnesses, on the other hand, can be dealt with at any time. Off-games chits can be issued at morning surgery ...
Hey-o all, I was just wondering what kind of painting programs people like? Whats a good program for simple things (like making avatars & game chits)? Any software to avoid?
Peronist Argentine President Cristina Kirchner had a face-to-face chit chat with Fidel Castro today, and told the Governments press agency that Castro...
114) 多数の重症合併症を併発するも救命し得た急性感染性心内膜炎の一例(第189回日本循環器学会関東甲信越地方会) (2004 ...
時間に依存するファジィ集合族を用いた最適制御に関する一考察 (函数解析学の応用としての情報数理の研究) (2001 ...
There are 3 forms of beta-hexosaminidase: hexosaminidase A is a trimer, with one alpha, one beta-A and one beta-B chain; ... hexosaminidase B is a tetramer of two beta-A and two beta-B chains; and hexosaminidase S is a homodimer of alpha chains. The ... Glycoside hydrolase family 20 CAZY GH_20 comprises enzymes with several known activities; beta-hexosaminidase (EC 3.2.1.52); ... In the brain and other tissues, beta-hexosaminidase A degrades GM2 gangliosides; specifically, the enzyme hydrolyses terminal ...
OGA is a member of the family of hexosaminidases. However, unlike lysosomal hexosaminidases, OGA activity is the highest at ... For both types of inhibitors, OGA can be selected apart from the generic lysosomal hexosaminidases by elongating the C2 ... Alonso J, Schimpl M, van Aalten DM (December 2014). "O-GlcNAcase: promiscuous hexosaminidase or key regulator of O-GlcNAc ...
Kaback, Michael M. "Hexosaminidase A Deficiency". GeneReviews. Retrieved 2007-05-11. Machácková, Eva (2003). "Disease-causing ... Enzyme assay techniques detect individuals with lower levels of hexosaminidase A. Development of a serum enzyme assay test made ... 1993). "A second mutation associated with apparent beta-hexosaminidase A pseudodeficiency: identification and frequency ...
... a novel hexosaminidase. Production by Bacillus subtilis B, purification and characterization". European Journal of Biochemistry ...
Beta-hexosaminidase is a vital hydrolytic enzyme, found in the lysosomes, that breaks down lipids. When beta-hexosaminidase is ... This protein is a cofactor that is required for the normal function of beta-hexosaminidase A. The disease is usually fatal by ... Detection of heterozygotes and homozygotes by serum hexaminidase assay". New England Journal of Medicine. 283 (1): 15-20. doi: ... GeneReview/NIH/UW entry on Hexosaminidase A Deficiency. ... that result from a deficiency of the enzyme beta-hexosaminidase ...
He also proved that Tay-Sachs disease patients could be diagnosed by enzyme assay of hexosaminidase A. Further development of ... Detection of heterozygotes and homozygotes by serum hexaminidase assay". New England Journal of Medicine. 283 (1): 15-20. doi: ... enzyme assay testing demonstrated that levels of both hexosaminidases A and B could be measured in patients and carriers, ...
... beta-hexosaminidase. AB variant is caused by a failure in the gene that makes an enzyme cofactor for beta-hexosaminidase, ... except that there is deficiency of both hexosaminidase A and hexosaminidase B. Infants with this disorder typically appear ... This protein is required for the normal function of beta-hexosaminidase A, a critical enzyme in the nervous system that breaks ... If mutations in both alleles at this locus disrupt the activity of the GM2 activator, beta-hexosaminidase A cannot perform its ...
Hexosaminidase A (alpha polypeptide), also known as HEXA, is an enzyme that in humans is encoded by the HEXA gene, located on ... Hexosaminidase A is a heterodimer composed of an alpha subunit (this protein) and a beta subunit. The alpha subunit polypeptide ... Hexosaminidase A at the US National Library of Medicine Medical Subject Headings (MeSH) EC 3.2.1.52 National Tay-Sach's Disease ... Hexosaminidase A and the cofactor GM2 activator protein catalyze the degradation of the GM2 gangliosides and other molecules ...
As a member of family 20 β-hexosaminidases, it cleaves terminal monosaccharide residues from the non-reducing end of the ... Dispersin B is a β-hexosaminidase that specifically hydrolyzes β-1,6-glycosidic linkages of acetylglucosamine polymers found in ... Williams SJ, Mark BL, Vocadlo DJ, James MN, Withers SG (2002). "Aspartate 313 in the Streptomyces plicatus hexosaminidase plays ... "The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease". J Mol Biol. 328 (3): ...
The gene provides instructions for making a protein crucial to the enzymes beta-hexosaminidase A and beta-hexosaminidase B, ... hexosaminidase activity was almost completely absent. The disease causing catabolic enzyme deficiency of hexosaminidases was ... In particular, beta-hexosaminidase A breaks down a fatty compound called GM2 ganglioside. Mutations in the HEXB gene disrupt ... Kytzia HJ, Hinrichs U, Maire I, Suzuki K, Sandhoff K (1983). "Variant of GM2-gangliosidosis with hexosaminidase A having a ...
Total hexosaminidase enzyme activity is decreased in individuals with Tay-Sachs as is the percentage of hexosaminidase A. After ... Tay-Sachs disease is caused by insufficient activity of the enzyme hexosaminidase A. Hexosaminidase A is a vital hydrolytic ... which codes for a subunit of the hexosaminidase enzyme known as hexosaminidase A. It is inherited from a person's parents in an ... When hexosaminidase A is no longer functioning properly, the lipids accumulate in the brain and interfere with normal ...
"Detachment of Actinobacillus actinomycetemcomitans biofilm cells by an endogenous beta-hexosaminidase activity". Journal of ...
"Pyrimethamine increases β-hexosaminidase A activity in patients with Late Onset Tay Sachs". Molecular Genetics and Metabolism. ... researchers discovered that pyrimethamine can increase β-hexosaminidase activity, thus potentially slowing down the progression ...
Beta-hexosaminidase subunit beta is an enzyme that in humans is encoded by the HEXB gene. Hexosaminidase B is the beta subunit ... Beta-hexosaminidase is composed of two subunits, alpha and beta, which are encoded by separate genes. Both beta-hexosaminidase ... Intracerebroventricular administration of the modified β-hexosaminidase B to Sandhoff mode mice restored the β-hexosaminidase ... "Entrez Gene: HEXB hexosaminidase B (beta polypeptide)". Bateman KS, Cherney MM, Mahuran DJ, Tropak M, James MN (March 2011). " ...
1993). "A second mutation associated with apparent beta-hexosaminidase A pseudodeficiency: identification and frequency ...
This protein binds molecules of ganglioside GM2, extracts them from membranes, and presents them to beta-hexosaminidase A for ... Gangliosidosis Sandhoff disease Tay-Sachs disease Hexosaminidase GM1 GRCh38: Ensembl release 89: ENSG00000196743 - Ensembl, May ... β-hexosaminidase A, together with GM2 ganglioside activator, catalyzes the degradation of the ganglioside GM2, and other ... GM2 ganglioside and hexosaminidase A". Biochim. Biophys. Acta. 1340 (1): 45-52. doi:10.1016/S0167-4838(97)00027-7. PMID 9217013 ...
Lysosomal exocytosis is typically evaluated by estimation of released lysosomal enzymes such as beta hexosaminidase. An ...
Beta-hexosaminidases are composed of either a HexA/HexB heterodimer or a HexB homodimer, and can hydrolyse diverse substrates, ... Mark BL, Mahuran DJ, Cherney MM, Zhao D, Knapp S, James MN (April 2003). "Crystal structure of human beta-hexosaminidase B: ... In molecular biology, the CHB HEX N-terminal domain represents the N-terminal domain in chitobiases and beta-hexosaminidases. ...
Some lysosomal enzymes are elevated, including tartrate-resistant acid phosphatase, hexosaminidase, and a human chitinase, ...
Lemansky P, Gieselmann V, Hasilik A, von Figura K (August 1984). "Cathepsin D and beta-hexosaminidase synthesized in the ...
On top of that, M. pneumoniae induces the activation of mast cells by releasing serotonin and hexosaminidase. By producing ...
"Crystallographic Structure of Human β-Hexosaminidase A: Interpretation of Tay-Sachs Mutations and Loss of GM2 Ganglioside ...
Hexosaminidase A (alpha polypeptide), also known as HEXA, is an enzyme that in humans is encoded by the HEXA gene, located on ... Hexosaminidase A and the cofactor GM2 activator protein catalyze the degradation of the GM2 gangliosides and other molecules ... Even though the alpha and beta subunits of hexosaminidase A can both cleave GalNAc residues, only the alpha subunit is able to ... Proia RL, Soravia E (April 1987). "Organization of the gene encoding the human beta-hexosaminidase alpha-chain". The Journal of ...
Ohno, Kousaku & Suzuki, Kunihiko (1988-12-05). "Multiple Abnormal beta-Hexosaminidase alpha-Chain mRNAs in a Compound- ...
The HEXA gene makes part of an enzyme called beta-hexosaminidase A, which plays a critical role in the nervous system. This ... Mutations in the HEXA gene disrupt the activity of beta-hexosaminidase A, preventing the breakdown of the fatty substances. As ...
HEXA: hexosaminidase A (alpha polypeptide)(Tay-Sachs disease). *HMG20A: encoding protein High mobility group protein 20A ...
... in which patients lack the enzyme hexosaminidase. One example of enzyme deficiency is the most common type of phenylketonuria. ...
... a hexosaminidase) from a soil organism. Treating the polysaccharide with this enzyme abrogated its serological reactivity. ...
As PUGNAc also inhibits lysosomal β-hexosaminidases, the OGA-selective inhibitor NButGT was developed to further probe the ...
For example, the fatal Tay-Sachs disease arises as a genetic defect which leads to no functional hexosaminidase A produced, ...
N-acetyl-beta-D-hexosaminidase, N-acetyl-beta-hexosaminidase, N-acetyl hexosaminidase, beta-hexosaminidase, beta- ... GeneReviews/NCBI/NIH/UW entry on hexosaminidase A deficiency, Tay-Sachs disease hexosaminidase A at the US National Library of ... NCOAT is also known as hexosaminidase C and has distinct substrate specificities compared to lysosomal hexosaminidase A. A ... The GM2 activator protein transports GM2 gangliosides and presents the lipids to hexosaminidase, so a functional hexosaminidase ...
Beta-hexosaminidase A (HEX A) enzymatic activity. Affected individuals. The diagnosis of hexosaminidase A deficiency relies on ... Juvenile (subacute) hexosaminidase A deficiency. Juvenile hexosaminidase A deficiency often begins with ataxia and ... Beta-hexosaminidase subunit alpha. alsod/HEXA genetic mutations. HEXA homepage - Mendelian genes. Hexosaminidase A; Tay-Sachs ... Hexosaminidase A Deficiency. Synonyms: HEX A Deficiency, GM2 Gangliosidosis. Includes: Chronic and Adult-Onset Hexosaminidase A ...
IPR022956 Beta_hexosaminidase_bac. IPR019800 Glyco_hydro_3_AS. IPR001764 Glyco_hydro_3_N. IPR036962 Glyco_hydro_3_N_sf. ... IPR022956 Beta_hexosaminidase_bac. IPR019800 Glyco_hydro_3_AS. IPR001764 Glyco_hydro_3_N. IPR036962 Glyco_hydro_3_N_sf. ... Beta-hexosaminidaseUniRule annotation. ,p>Information which has been generated by the UniProtKB automatic annotation system, ... tr,A0A0H5ZA02,A0A0H5ZA02_VIBCL Beta-hexosaminidase OS=Vibrio cholerae OX=666 GN=nagZ PE=3 SV=1 ...
... hexosaminidase explanation free. What is hexosaminidase? Meaning of hexosaminidase medical term. What does hexosaminidase mean? ... Looking for online definition of hexosaminidase in the Medical Dictionary? ... Standardization of leukocytes for Tay-Sachs hexosaminidase assay [Abstract].. Automated determination of serum hexosaminidase A ... hexosaminidase. Also found in: Dictionary, Encyclopedia, Wikipedia. hex·os·a·min·i·dase. (heksōs-ă-mini-dās), General term ...
There are 3 forms of beta-hexosaminidase: hexosaminidase A is an heterodimer composed of one subunit alpha and one subunit beta ... chain A and B); hexosaminidase B is an homodimer of two beta subunits (two chains A and B); hexosaminidase S is a homodimer of ... Beta-hexosaminidase subunit betaAdd BLAST. 514. Amino acid modifications. Feature key. Position(s). DescriptionActions. ... hexosaminidase activity Source: RGDInferred from direct assayi*. "Rat brain contains high levels of mannose-6-phosphorylated ...
Aspartate 313 in the Streptomyces plicatus hexosaminidase plays a critical role in substrate-assisted catalysis by orienting ... Wildtype Streptomyces plicatus beta-hexosaminidase in complex with product (GlcNAc). *DOI: 10.2210/pdb1m01/pdb ...
Shop a large selection of products and learn more about Hexosaminidase A/HEXA Mouse anti-Human, Alexa Fluor 350, Clone: 20F1, ... beta-hexosaminidase subunit alpha, Beta-N-acetylhexosaminidase subunit alpha, EC 3.2.1, EC 3.2.1.52, hexosaminidase A (alpha ... Hexosaminidase A/HEXA Monoclonal antibody specifically detects Hexosaminidase A/HEXA in Human samples. It is validated for ... Hexosaminidase A/HEXA Mouse anti-Human, Alexa Fluor 350, Clone: 20F1, Novus Biologicals ...
TSD, hexosaminidase A, Beta-hexosaminidase subunit alpha, Beta-N-acetylhexosaminidase subunit alpha, Hexosaminidase subunit A, ... HEXA is the alpha subunit of the lysosomal enzyme beta-hexosaminidase which, combined with the cofactor GM2 activator protein, ... The two subunits composing Beta-hexosaminidase, alpha and beta, belong to the glycosyl hydrolases family and are encoded by ...
Aspartate 313 in the Streptomyces plicatus hexosaminidase plays a critical role in substrate-assisted catalysis by orienting ...
Author Summary Phagocytosis is the cellular process of engulfing solid particles to form an internal phagosome in protozoa, algae, and professional phagocytes of multicellular eukaryotic organisms. In phagocytic protozoa, phagocytosis is involved in the acquisition of nutrients, and the evasion from the host immune system and inflammation. While hydrolytic enzymes that are essential for the efficient and regulated degradation of phagocytosed particles, such as bacteria, fungi, and eukaryotic organisms, have been characterized, the mechanisms of the transport of these proteins are poorly understood. In the present study, we have demonstrated, for the first time, the molecular mechanisms of how the digestive enzymes are transported to phagosomes. Understanding of such mechanisms of the transport of phagosomal proteins at the molecular level may lead to the identification of a novel target for the development of new preventive measures against parasitic infections caused by phagocytic protozoa.
This website uses cookies to provide you the best browsing experience. By continuing to use this website you accept our use of cookies.. ...
An Attempt to Purify N-Acetyl-β-hexosaminidases from Crude Extracts of Human Liver by Affinty Chromatography ACHAMMA KOSHY; ... ACHAMMA KOSHY, DONALD ROBINSON, JOHN L. STIRLING; An Attempt to Purify N-Acetyl-β-hexosaminidases from Crude Extracts of Human ...
b-Hexosaminidase. Principles of Method: Total hexosaminidase is measured from the hydrolysis of the synthetic substrate 4- ... The hexosaminidases are composed of two different subunits, a and b, coded by genes HEXA and HEXB. Hex A is ab and hex B is bb ... which releases fluorescent 4-methylumbelliferone when acted upon by a b-hexosaminidase. Hexosaminidase A (hex A) is determined ... b-hexosaminidase (total and A) is elevated in plasma and serum from patients with I-cell disease and pseudo-Hurler ...
Purification of normal human urinary N-acetyl-β-hexosaminidase A by affinity chromatography. D K Banerjee, D Basu ... N-Acetyl-beta-hexosaminidase A was purified 1000-fold from human urine by chromatography on DEAE-Sephadex followed by ... Purification of normal human urinary N-acetyl-β-hexosaminidase A by affinity chromatography ... Purification of normal human urinary N-acetyl-β-hexosaminidase A by affinity chromatography ...
Hydrolysis of Tay-Sachs ganglioside by beta-hexosaminidase A of human liver and urine ... Characterization of beta d n acetyl hexosaminidase c and beta d n acetyl hexosaminidase s in fibroblasts from control ... I. Antigenic differences between hexosaminidase A and hexosaminidase B. International Archives of Allergy and Applied ... Isolation of tay sachs ganglioside from roe of striped mullet mugil cephalus and its degradation by human beta hexosaminidase. ...
What is the meaning of HEXOSAMINIDASE? How do you use HEXOSAMINIDASE in a sentence? What are synonyms for HEXOSAMINIDASE? ...
Deficiency of hexosaminidase B due to mutations in the gene encoding the hexosaminidase beta subunit is a case of SANDHOFF ... A mammalian beta-hexosaminidase isoform that is comprized of hexosaminidase beta subunits. ... Hexosaminidase B [Chemical/Ingredient] A mammalian beta-hexosaminidase isoform that is comprized of hexosaminidase beta ... beta-Hexosaminidase B-isoforms (beta-hexosaminidase B, P, and intermediate forms; abbreviated herein as Hex B) and serum… ( ...
This is due to a deficient enzyme, β-hexosaminidase A (HEXA), involved in the catabolism of GM2 gangliosides. A recently ... This successful use of the R&P and scAAV9-HEXM resulted in significantly higher hexosaminidase enzyme activity levels in serum ... Induction of Immune Tolerance to Hexosaminidase M Using Rapamycin and Prednisone in the scAAV9-HEXM Gene Therapy Treatment of ... Induction of Immune Tolerance to Hexosaminidase M Using Rapamycin and Prednisone in the scAAV9-HEXM Gene Therapy Treatment of ...
... which is further processed by β-hexosaminidase B to lactosyl-ceramide, thereby bypassing the β-hexosaminidase A defect. Since ... which is further processed by β-hexosaminidase B to lactosyl-ceramide, thereby bypassing the β-hexosaminidase A defect. Since ... Hexa−/− mice, depleted of β-hexosaminidase A, remain asymptomatic to 1 year of age, because they catabolise GM2 ganglioside via ... Hexa−/− mice, depleted of β-hexosaminidase A, remain asymptomatic to 1 year of age, because they catabolise GM2 ganglioside via ...
Lysosomal beta-hexosaminidase A (Hex A) is essential for the degradation of G(M2) gangliosides in the central and peripheral ... Crystallographic structure of human beta-hexosaminidase A: Interpretation of Tay-Sachs mutations and loss of G(M2) ganglioside ... Crystallographic structure of human beta-hexosaminidase A: Interpretation of Tay-Sachs mutations and loss of G(M2) ganglioside ... Crystallographic structure of human beta-hexosaminidase A: Interpretation of Tay-Sachs mutations and loss of G(M2) ganglioside ...
Aspartate 313 in the Streptomyces plicatus hexosaminidase plays a critical role in substrate-assisted catalysis by orienting ... Aspartate 313 in the Streptomyces plicatus hexosaminidase plays a critical role in substrate-assisted catalysis by orienting ... Aspartate 313 in the Streptomyces plicatus hexosaminidase plays a critical role in substrate-assisted catalysis by orienting ... the roles played by the Asp-313 residue is likely general for all hexosaminidases using a mechanism involving substrate- ...
Phylogenetic analysis of insect β-N-acetyl-D-hexosaminidases and vertebrate β-N-acetyl-D-hexosaminidases. The GenBank ID of the ... In addition to the above-mentioned β-N-acetyl-D-hexosaminidases, another, broad substrate-spectrum β-N-acetyl-D-hexosaminidase ... The X-ray crystal structure of human β-hexosaminidase B provides new insights into Sandhoff disease. J Mol Biol. 2003;328:669- ... A single site in human β-hexosaminidase A binds both 6-sulfate-groups on hexosamines and the sialic acid moiety of GM2 ...
What is Beta-hexosaminidase? Meaning of Beta-hexosaminidase medical term. What does Beta-hexosaminidase mean? ... Looking for online definition of Beta-hexosaminidase in the Medical Dictionary? Beta-hexosaminidase explanation free. ... hexosaminidase. (redirected from Beta-hexosaminidase). Also found in: Dictionary. hex·os·a·min·i·dase. (heksōs-ă-mini-dās), ... hexosaminidase. /hex·os·amin·i·dase/ (hek″sōs-ah-min´ĭ-dās) 1. any of the enzymes that cleave hexosamines or acetylated ...
... of hexosaminidase S, and the much higher K(m) and lower pH optimum of hexosaminidase B, toward sulfated versus unsulfated ... In mammals beta-hexosaminidase B (betabeta) and S (alphaalpha) are other major and minor isozymes. The primary structures of ... Hexosaminidase has been grouped with glycosidases in family 20. A molecular model of the active site of the human ... Neutralization of the charge on alphaArg(424) by substituting Gln produced a hexosaminidase S with a K(m) decrease of 3-fold ...
Zellfunktionen: intrazelluläre Ca2+-Mobilisierung (über Fura-2-Fluoreszenz) und Exozytose (beta-Hexosaminidase-Freisetzung; ... Intracellular Ca Mobilization and Beta-hexosaminidase Release Are Not Influenced by 60 Hz-electromagnetic Fields (EMF) in RBL ... Intrazelluläre Kalzium-Mobilisierung und Beta-Hexosaminidase-Freisetzung werden nicht von 60 Hz-elektromagnetischen Feldern ( ... Hexosaminidase-Freisetzung in diesen Zellen. Die Ergebnisse deuten darauf hin, dass die Magnetfeld-Exposition (60 Hz, 0.1 or 1 ...
Microheterogeneity of Serum β-Hexosaminidase in Chronic Alcohol Abusers in a Drivers License Regranting Program. ... In this study, we explore the microheterogeneity of β-hexosaminidase (β-HEX) in chronic alcohol abusers in the framework of a ...
What is a beta hexosaminidase test? A beta hexosaminidase test is a blood test performed in children to diagnose an inherited ... A routine blood draw is required in order to perform the beta hexosaminidase test. The blood sample is then taken to a ... Also known as: lysosomal for Tay Sachs, hexosaminidase A, total leukocytes/molecular reflex. ... Tay-Sachs disease is related to a deficiency of the beta-hexosaminidase enzyme. ...
Hexosaminidase A and B answers are found in the Daviss Lab & Diagnostic Tests powered by Unbound Medicine. Available for ... 456-592 nmol/hr/mL or 55-76% of total hexosaminidase. 7.62-9.88 units/L or 55-76% of total hexosaminidase. ... Hexosaminidase A and B is a topic covered in the Daviss Lab & Diagnostic Tests. To view the entire topic, please sign in or ... "Hexosaminidase a and B." Daviss Lab & Diagnostic Tests, 7th ed., F.A. Davis Company, 2017. Nursing Central, nursing. ...
... hexosaminidase A and B deficiency. Information about Beta Hex Deficiency ... Cashman NR, Antel JP, Hancock LW, et al; N-acetyl-beta-hexosaminidase beta locus defect and juvenile motor neuron disease: a ... Alpha subunits of b-hexosaminidase A: absence or defects of these results in Tay-Sachs disease (TSD) and its variants. ... Where there are abnormal beta chains both hexosaminidase A and B will be affected. With combined enzyme deficiency, there is ...
  • Total hexosaminidase is measured from the hydrolysis of the synthetic substrate 4-methylumbelliferyl-N-acetyl- a -D-glucosamine, which releases fluorescent 4-methylumbelliferone when acted upon by a b -hexosaminidase. (sas-centre.org)
  • Hex A is the deficient enzyme in Tay-Sachs disease and variants, whereas total hexosaminidase deficiency (hex A plus hex B) occurs in Sandhoff disease and variants. (sas-centre.org)
  • Assay of total hexosaminidase and of hex A (towards MUGS) is included in our lysosomal enzyme screening procedure for patients with a neurodegenerative disorder. (sas-centre.org)
  • Testing for Tay-Sachs and Sandhoff diseases occurs by analysis of hexosaminidase A, a heat-labile enzyme, and total hexosaminidase (hexosaminidase A plus hexosaminidase B). When testing the enzyme, an artificial substrate is most commonly used. (testcatalog.org)
  • The total hexosaminidase is quantified. (testcatalog.org)
  • Biochemically, Tay-Sachs disease is characterized by normal total hexosaminidase with a very low percent hexosaminidase A. Carriers of Tay-Sachs disease are asymptomatic, but have intermediate percent hexosaminidase A in serum and leukocytes. (testcatalog.org)
  • Heterozygotes for the disease are usually identified by their relatively low ratio of heat-labile HEX A to total hexosaminidase. (biomedsearch.com)
  • Biochemical criteria included deficiency of hexosaminidase A (less than 2% of total hexosaminidase activity for infantile patients). (nih.gov)
  • β- N -acetyl-D-hexosaminidases (EC 3.2.1.52) belong to the glycoside hydrolase family 20 and catalyze the removal of N- acetyl-D-glucosamine (GlcNAc) or N- acetyl-D-galactosamine (GalNAc) from the non-reducing ends of a variety of physiological substrates, such as oligosaccharides, glycoproteins and glycolipids. (ijbs.com)
  • Hexosaminidase A deficiency results in a group of neurodegenerative disorders caused by intralysosomal storage of the specific glycosphingolipid, GM2 ganglioside. (archive.org)
  • 2. a specific hexosaminidase acting on keratan sulfate and ganglioside GM2 and related compounds. (thefreedictionary.com)
  • HEXA is the alpha subunit of the lysosomal enzyme beta-hexosaminidase which, combined with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules having N-acetyl hexosamines terminus. (prospecbio.com)
  • Tay-Sachs disease is a severe lysosomal disorder caused by mutations in the HexA gene coding for the α-subunit of lysosomal β-hexosaminidase A, which converts G M2 to G M3 ganglioside. (prolekare.cz)
  • Hexa −/− mice, depleted of β-hexosaminidase A, remain asymptomatic to 1 year of age, because they catabolise G M2 ganglioside via a lysosomal sialidase into glycolipid G A2 , which is further processed by β-hexosaminidase B to lactosyl-ceramide, thereby bypassing the β-hexosaminidase A defect. (prolekare.cz)
  • The disorder is caused by mutations in the HexA gene coding for the α-subunit of lysosomal β-hexosaminidase A (HexA), which removes N-acetyl-glucosamine residue from G M2 ganglioside, converting it to G M3 ganglioside. (prolekare.cz)
  • The physiological importance of human β- N -acetyl-D-hexosaminidase A (HsHex A, αβ heterdimer) and B (HsHexB, ββ homodimer) are linked to the degradation of glycoconjugates (such as GM2 ganglioside) [ 12 , 13 ]. (ijbs.com)
  • In humans, beta-hexosaminidase A (alphabeta) is required to hydrolyze GM2 ganglioside. (semanticscholar.org)
  • Construction of a hybrid β-hexosaminidase subunit capable of forming stable homodimers that hydrolyze GM2 ganglioside in vivo. (semanticscholar.org)
  • Within lysosomes, beta-hexosaminidase A forms part of a complex that breaks down a fatty substance called GM2 ganglioside. (medlineplus.gov)
  • These mutations reduce or eliminate the activity of the enzyme beta-hexosaminidase A, which prevents the enzyme from breaking down GM2 ganglioside. (medlineplus.gov)
  • Within lysosomes, beta-hexosaminidase A helps break down a fatty substance called GM2 ganglioside. (medlineplus.gov)
  • Mutations in the HEXA gene disrupt the activity of beta-hexosaminidase A, which prevents the enzyme from breaking down GM2 ganglioside. (medlineplus.gov)
  • Sandhoff disease (SD) is a lysosomal storage disease, caused by loss of beta-hexosaminidase (HEX) activity resulting in the accumulation of ganglioside GM2. (umassmed.edu)
  • The restoration/overexpression of β-Hexosaminidase leads to the reduction of intracellular GM2 ganglioside storage in transduced and in cross-corrected SD murine neural progeny, indicating that the transgenic enzyme is secreted and functional. (unipg.it)
  • The diagnosis of hexosaminidase A deficiency relies on the demonstration of absent to near-absent beta-hexosaminidase A (HEX A) enzymatic activity in the serum or white blood cells of a symptomatic individual in the presence of normal or elevated activity of the beta-hexosaminidase B (HEX B) isoenzyme. (archive.org)
  • Increases and time-course variations in beta-hexosaminidase isoenzyme B and carbohydrate-deficient transferrin in serum from alcoholics are similar. (semanticscholar.org)
  • A recently constructed isoenzyme analogous to HEXA, called Hexosaminidase M (HEXM), can efficiently catabolize GM2 gangliosides, while its gene can be compactly packaged into the self-complementary adeno-associated viral vector serotype 9 (scAAV9). (queensu.ca)
  • Comparison of serum beta-hexosaminidase isoenzyme B activity with serum carbohydrate-deficient transferrin and other markers of alcohol abuse. (thefreedictionary.com)
  • A fourth mammalian hexosaminidase polypeptide which has been designated hexosaminidase D (HEXDC) has recently been identified. (wikipedia.org)
  • The subunit and polypeptide structure of hexosaminidase from human placenta. (thefreedictionary.com)
  • The hexosaminidases are composed of two different subunits, a and b , coded by genes HEXA and HEXB . (sas-centre.org)
  • The clinically similar disorder, Sandhoff disease is caused by the mutations in the HexB gene coding for the β-subunit of hexosaminidase A which results in simultaneous deficiency of Hex A and HexB [1] . (prolekare.cz)
  • Sandhoff disease is an autosomal recessive condition resulting from 2 mutations in HEXB , which encodes for the beta subunit of hexosaminidase. (testcatalog.org)
  • Tay-Sachs and Sandhoff disease (the main forms of GM2 gangliosidosis) result from mutations in either the HEXA or HEXB genes encoding, respectively, the α- or β-subunits of the lysosomal β-Hexosaminidase enzyme. (unipg.it)
  • In physiological conditions, α- and β-subunits combine to generate β-Hexosaminidase A (HexA, αβ) and β-Hexosaminidase B (HexB, ββ). (unipg.it)
  • Overall, these results confirm the potential of P. pastoris as host to produce recombinant β-hexosaminidases intended to be used in the treatment of GM2 gangliosidosis. (nih.gov)
  • 1996. Mice lacking both subunits of lysosomal beta-hexosaminidase display gangliosidosis and mucopolysaccharidosis. (jax.org)
  • Expression of hexosaminidase isoenzymes in childhood leukemia. (thefreedictionary.com)
  • Hexosaminidase A (hex A) is determined as a percentage figure from the amount of activity left after samples are heated for 3h at 50° C. This procedure leads to loss of hex A which is heat-labile but not of hex B or intermediate isoenzymes. (sas-centre.org)
  • In humans, two major β-hexosaminidase isoenzymes exist: Hex A and Hex B. Hex A is a heterodimer of subunits α and β (60% identity), whereas Hex B is a homodimer of β-subunits. (researchwithrutgers.com)
  • Functional lysosomal β-hexosaminidase enzymes are dimeric in structure. (wikipedia.org)
  • At least four specific enzymes carrying out this type of reaction are known: α- N -acetyl- d -galactosaminidase, α- N -acetyl- d -glucosaminidase, β- N -acetyl- d -hexosaminidase, and β- N -acetyl- d -galactosaminidase, each being specific for the configuration and type of sugar included in the name. (thefreedictionary.com)
  • In preliminary experiments with other lysosomal enzymes, such as hexosaminidase and [beta]-galactosidase, we observed that activities on filter-paper cards were stable over a period of several weeks when stored dry at room temperature. (thefreedictionary.com)
  • Insect β- N -acetyl-D-hexosaminidases with broad substrate-spectrum (IBS-Hex) are the homologues of human β- N -acetyl-D-hexosaminidase A/B (HsHex A/ B). These enzymes are distributed in most insect species and vary in physiological roles. (ijbs.com)
  • IBS-Hexs are found to have high sequence similarity to human β- N -acetyl-D-hexosaminidases but low sequence similarity with other insect Hex enzymes. (ijbs.com)
  • This test measures the activity of a group of enzymes known as hexosaminidases and the specific enzyme beta-hexosaminidase A in blood. (adam.com)
  • Tay-Sachs and Sandhoff diseases are lysosomal storage disorders, also referred to as GM2 gangliosidoses, caused by deficiencies of the enzymes hexosaminidase A and hexosaminidase B, respectively. (testcatalog.org)
  • Geiger B, Arnon R, Sandhoff IK Immunochemical and Biochemical Investigation of Hexosaminidase S, The American Journal of Human Genetics, 29: 508-522, 1977. (thefreedictionary.com)
  • Crystal structure of human beta-hexosaminidase B: understanding the molecular basis of Sandhoff and Tay-Sachs disease. (semanticscholar.org)
  • Individuals with Sandhoff disease have deficiencies in both hexosaminidase A and hexosaminidase B. Phenotypically, patients with Sandhoff disease present with features very similar to Tay-Sachs disease including variability in age of onset and severity. (testcatalog.org)
  • Adult Tay-Sachs disease and Sandhoff diseases are caused by deficiency of an enzyme called β-hexosaminidase A, or Hex A in short. (clinicaltrials.gov)
  • Adult Tay-Sachs (TSD) and Sandhoff disease (SD) result from a deficiency of lysosomal heterodimeric β-hexosaminidase A (Hex A, αβ). (clinicaltrials.gov)
  • Moreover, docking in combination with consequent molecular dynamics simulations of C-6 modified glycosides enabled us to identify the structural features required for accommodation and processing of these bulky substrates in the active site of hexosaminidase from T. flavus. (nih.gov)
  • In case of the sulfated substrate 6 docking into the active site of hexosaminidase from S. plicatus was successful only when applying flexibility to the amino acid residues. (nih.gov)
  • NCOAT is also known as hexosaminidase C and has distinct substrate specificities compared to lysosomal hexosaminidase A. A single-nucleotide polymorphism in the human O-GlcNAcase gene is linked to diabetes mellitus type 2. (wikipedia.org)
  • A substrate specific cofactor of beta-hexosaminidase A. (semanticscholar.org)
  • Based on analogous conserved residues in the family 18 chitinases and family 56 hyaluronidases, the roles played by the Asp-313 residue is likely general for all hexosaminidases using a mechanism involving substrate-assisted catalysis. (ubc.ca)
  • Liu F, Liu T, Qu M, Yang Q. Molecular and Biochemical Characterization of a Novel β-N-Acetyl-D-Hexosaminidase with Broad Substrate-Spectrum from the Aisan Corn Borer, Ostrinia Furnacalis . (ijbs.com)
  • In insects, two β- N -acetyl-D-hexosaminidase isoforms with strict substrate spectra have been extensively studied. (ijbs.com)
  • In addition to the above-mentioned β- N -acetyl-D-hexosaminidases, another, broad substrate-spectrum β- N -acetyl-D-hexosaminidase (IBS-Hex) can be found in insects. (ijbs.com)
  • Primary sequence analysis revealed some variable regions able to influence difference in substrate affinity of hexosaminidases. (nih.gov)
  • Active site of hexosaminidases with docked pNP-GlcNAc-sulfate 6 after molecular dynamics simulation with shown residues at the distance less than 0.3 nm from the sulfate group of the substrate. (nih.gov)
  • Among the most promising research at this point in time is enzyme replacement therapy, the Jacob sheep model, substrate reduction therapy and the increasing the activity of β-hexosaminidase A. (news-medical.net)
  • Such sequence variations are not associated with disease, but result in the production of a hexosaminidase A enzyme with decreased activity towards the artificial substrate typically used in the enzyme assay. (testcatalog.org)
  • Even though the alpha and beta subunits of lysosomal hexosaminidase can both cleave GalNAc residues, only the alpha subunit is able to hydrolyze GM2 gangliosides because of a key residue, Arg-424, and a loop structure that forms from the amino acid sequence in the alpha subunit. (wikipedia.org)
  • There are numerous mutations that lead to hexosaminidase deficiency including gene deletions, nonsense mutations, and missense mutations. (wikipedia.org)
  • The two subunits of hexosaminidase A are shown below: The bifunctional protein NCOAT (nuclear cytoplasmic O-GlcNAcase and acetyltransferase) that is encoded by the MGEA5 gene possesses both hexosaminidase and histone acetyltransferase activities. (wikipedia.org)
  • HEXA (Hexosaminidase Subunit Alpha) is a Protein Coding gene. (genecards.org)
  • The HEXA gene provides instructions for making one part (subunit) of an enzyme called beta-hexosaminidase A. Specifically, the protein produced from the HEXA gene forms the alpha subunit of this enzyme. (medlineplus.gov)
  • This example uses MATLAB® and Bioinformatics Toolbox to find PCR primers for the human hexosaminidase gene. (mathworks.com)
  • The HEXA gene provides instructions for making part of an enzyme called beta-hexosaminidase A, which plays a critical role in the brain and spinal cord. (medlineplus.gov)
  • Tay Sachs disease (TSD) is a neurodegenerative disorder due to β-hexosaminidase A deficiency caused by mutations in the HEXA gene. (nih.gov)
  • Expression of human lysosomal beta-hexosaminidase in yeast vacuoles, Biochemical and Biophysical Research Communications, 170: 383-90, 1990. (thefreedictionary.com)
  • Individuals with Tay-Sachs disease have a deficiency of hexosaminidase A. Variability is observed with respect to age of onset and clinical symptoms. (testcatalog.org)
  • Hexosaminidase A is a heterodimer composed of 1 alpha and 1 beta subunit (alpha-beta), while hexosaminidase B is a homodimer composed of 2 beta subunits (beta-beta). (testcatalog.org)
  • In contrast, tHGA was unable to attenuate the release of both β-hexosaminidase and tumor necrosis factor (TNF)-α. (portlandpress.com)
  • Beta-hexosaminidase and the cofactor GM2 activator protein catalyze the degradation of the GM2 gangliosides and other molecules containing terminal N-acetyl hexosamines. (wikipedia.org)
  • The GM2 activator protein transports GM2 gangliosides and presents the lipids to hexosaminidase, so a functional hexosaminidase enzyme is able to hydrolyze GM2 gangliosides into GM3 gangliosides by removing the N-acetylgalactosamine (GalNAc) residue from GM2 gangliosides. (wikipedia.org)
  • This is due to a deficient enzyme, β-hexosaminidase A (HEXA), involved in the catabolism of GM2 gangliosides. (queensu.ca)
  • Lysosomal beta-hexosaminidase A (Hex A) is essential for the degradation of G(M2) gangliosides in the central and peripheral nervous system. (ubc.ca)
  • Loop 2 in the vicinity of the sulfate is marked by yellow dots: A. S. plicatus hexosaminidase B. TfHex. (nih.gov)
  • Here, we present the X-ray crystallographic structure of Hex A to Å resolution and the Cited by: Hexosaminidases are involved in important biological processes catalyzing the hydrolysis of N-acetyl-hexosaminyl residues in glycosaminoglycans and glycoconjugates. (awordathought.com)
  • Abstract Chitinolytic β-N-acetyl-D-hexosaminidases, as a class of chitin hydrolysis enzyme in insects, are a potential species-specific target for developing environmentally-friendly pesticides. (duhnnae.com)
  • This successful use of the R&P and scAAV9-HEXM resulted in significantly higher hexosaminidase enzyme activity levels in serum, reduced GM2 accumulation in the brain, and an increase in biodistribution of the vector in the liver. (queensu.ca)
  • Hexosaminidase activity can be measured in serum, leukocytes, tears and cultivated fibroblasts. (patient.info)
  • Pyrimethamine is a drug that has shown some activity in this area but the overall efficacy is not high, as the activity of β-hexosaminidase A is still much lower than in unaffected populations. (news-medical.net)
  • Upregulating β-hexosaminidase activity in rodents prevents α-synuclein lipid associations and protects dopaminergic neurons from α-synuclein-mediated neurotoxicity. (ox.ac.uk)
  • This accumulation is due to deficiency in the activity of the β-hexosaminidases Hex-A or Hex-B, which are dimeric hydrolases formed by αβ or ββ subunits, respectively. (nih.gov)
  • Data regarding hexosaminidase activity in arthritis are limited to a few publications. (osteoporoza.pl)
  • RBL-2H3 cell line was used to investigate the anti-allergic effect of algal SP through the evaluation of β-hexosaminidase activity. (mdpi.com)
  • In this study, crude alkaloid derived from different parts of K. arborea was evaluated for their inhibitory activity on histamine and beta-hexosaminidase release in rat basophilic leukemic (RBL-2H3) cells. (tsijournals.com)
  • The effect of K. arborea on histamine, beta-hexosaminidase release and beta-hexosaminidase activity in RBL-2H3 cell was determined by using sandwich enzyme-linked immunosorbent assay (ELISA). (tsijournals.com)
  • Meanwhile, crude alkaloid extracted from K. arborea (roots) exhibited the highest inhibition of beta-hexosaminidase release and activity than ketotifen fumarate. (tsijournals.com)
  • Upregulating beta-hexosaminidase activity in rodents prevents alpha-sy" by Oeystein R. Brekk, Joanna A. Korecka et al. (umassmed.edu)
  • Tay-Sachs disease results from a loss of activity of hexosaminidase A (HEXA) in body tissues and fluids. (biomedsearch.com)
  • We show that these LVs drive the safe and coordinate expression of the α- and β-subunits, leading to supranormal levels of β-Hexosaminidase activity with prevalent formation of a functional HexA in SD murine neurons and glia, murine bone marrow-derived hematopoietic stem/progenitor cells (HSPCs), and human SD fibroblasts. (unipg.it)
  • Total leukocyte hexosaminidase activity was assayed by 4-methylumbelliferyl-N-acetyl-β-D-glucosamine lysis and hexosaminidase A activity was assayed by heat inactivation method and 4-methylumbelliferyl-N-acetyl-β-D-glucosamine-6-sulphate lysis method. (nih.gov)
  • IgE-mediated release of leukotriene C4, chondroitin sulfate E proteoglycan, beta-hexosaminidase , and histamine from cultured bone marrow-derived mouse mast cells. (thefreedictionary.com)
  • These results indicated that crude alkaloid from this plant consist of bioactive compounds which have the ability to inhibit mast cell degranulation via inhibition of histamine and beta-hexosaminidase release and have potential to provide as alternative anti-allergic agent. (tsijournals.com)
  • Activated mast cells release beta-hexosaminidase together with histamine. (tsijournals.com)
  • When an IgE-antigen binds with Fc ε RI, the receptor is activated, and a variety of biologically active mediators are released, causing allergic reactions, including the release of β -hexosaminidase, a common degranulation marker, histamine, arachidonic acid metabolites, and inflammatory cytokines [ 6 ]. (hindawi.com)
  • Human recombinant lysosomal β-Hexosaminidases produced in Pichia pastoris efficiently reduced lipid accumulation in Tay-Sachs fibroblasts. (nih.gov)
  • Previously, we reported the production of active human recombinant β-hexosaminidases (rhHex-A and rhHex-B) in the methylotrophic yeast Pichia pastoris. (nih.gov)
  • Hexosaminidase A/HEXA Monoclonal antibody specifically detects Hexosaminidase A/HEXA in Human samples. (fishersci.com)
  • N-Acetyl-beta-hexosaminidase A was purified 1000-fold from human urine by chromatography on DEAE-Sephadex followed by concanavalin A--Sepharose affinity chromatography. (biochemj.org)
  • Physiological substrates for human lysosomal beta -hexosaminidase S. (semanticscholar.org)
  • Isolation of cDNA clones coding for the beta subunit of human beta-hexosaminidase. (semanticscholar.org)
  • Furthermore, the residue pair N423-R424/ D452-L453 important for the specificity of human β- N -acetyl-D-hexosaminidase subunits α/β toward charged/ non-charged substrates was not conserved in OfHex2 and other IBS-Hexs. (ijbs.com)
  • Identification of the 6-sulfate binding site unique to alpha-subunit-containing isozymes of human beta-hexosaminidase. (semanticscholar.org)
  • lsoelectric focusing of crude extracts demonstrated that human colonic carcinomas contained a higher proportion of β-hexosaminidase B than β-hexosaminidase A, while normal human colonic mucosa contained a higher proportion of the A form of the enzyme. (nebraska.edu)
  • Pretlow, Thomas G. / β-Hexosaminidase lsozymes in Human Colonic Carcinoma . (nebraska.edu)
  • Most of the known HEXA mutations result in a completely nonfunctional version of beta-hexosaminidase A. These mutations cause the severe form of Tay-Sachs disease, which appears in infancy. (medlineplus.gov)
  • Immunologic release of beta-hexosaminidase and beta-glucuronidase from purified rat serosal mast cells. (thefreedictionary.com)
  • Beta-hexosaminidase, an essential enzyme responsible for glycoprotein metabolism in cell homeostasis, is stored in secretory granule of mast cells [ 5 ]. (tsijournals.com)
  • Inhibition of beta-hexosaminidase release is a key strategy to restrain the degranulation process of mast cells [ 6 - 8 ]. (tsijournals.com)
  • Automated determination of serum hexosaminidase A by pH inactivation for determination of Tay-Sachs disease heterozygotes. (thefreedictionary.com)
  • Following this, heat inactivation of hexosaminidase A occurs with a second measurement of the total enzyme level. (testcatalog.org)
  • The two subunits composing Beta-hexosaminidase, alpha and beta, belong to the glycosyl hydrolases family and are encoded by distinct genes. (prospecbio.com)
  • A mammalian beta-hexosaminidase isoform that is comprized of hexosaminidase beta subunits. (semanticscholar.org)
  • In mammals beta-hexosaminidase B (betabeta) and S (alphaalpha) are other major and minor isozymes. (semanticscholar.org)
  • Intracellular Ca Mobilization and Beta-hexosaminidase Release Are Not Influenced by 60 Hz-electromagnetic Fields (EMF) in RBL 2H3 Cells. (emf-portal.org)
  • Intrazelluläre Kalzium-Mobilisierung und Beta-Hexosaminidase-Freisetzung werden nicht von 60 Hz-elektromagnetischen Feldern (EMF) in RBL 2H3-Zellen beeinflusst. (emf-portal.org)
  • Es sollte die Wirkung elektromagnetischer Felder auf die intrazelluläre Ca 2+ -Mobilisierung (als universeller Botenstoff) untersucht werden, die durch Melittin (0.1-1 µM), Ionomycin (1-100 nM) und Thapsigargin (10-1000 nM) stimuliert wird, sowie auf die beta- Hexosaminidase -Freisetzung in RBL-2H3- Zellen . (emf-portal.org)
  • Die Magnetfeld - Exposition ( 60 Hz , 0,1 oder 1 mT) für 4 oder 16 h beeinflusste nicht die grundlegende oder 1 µM-Melittin- induzierte beta- Hexosaminidase -Freisetzung in diesen Zellen . (emf-portal.org)
  • What is a beta hexosaminidase test? (nicklauschildrens.org)
  • A beta hexosaminidase test is a blood test performed in children to diagnose an inherited disorder known as Tay-Sachs disease. (nicklauschildrens.org)
  • Tay-Sachs disease is related to a deficiency of the beta-hexosaminidase enzyme. (nicklauschildrens.org)
  • A routine blood draw is required in order to perform the beta hexosaminidase test. (nicklauschildrens.org)
  • Beta hexosaminidase deficiency. (patient.info)
  • Where there are abnormal beta chains both hexosaminidase A and B will be affected. (patient.info)
  • The encoded preproprotein is proteolytically processed to generate the alpha subunit of the lysosomal enzyme beta-hexosaminidase. (genecards.org)
  • Beta-hexosaminidase A plays a critical role in the brain and spinal cord (central nervous system). (medlineplus.gov)
  • Therefore, various anti-allergic studies involved the use of beta-hexosaminidase as a marker for mast cell degranulation in rat basophilic leukemia (RBL-2H3) cell line. (tsijournals.com)
  • In the beginning of the simulation of hexosaminidase from S. plicatus the binding energy of uronate 5 was favorable, however, during the simulation the interaction with catalytic Asp 313 and Glu 314 residues was lost. (nih.gov)
  • Hexosaminidase (Hex) cleaves terminal N-acetylglucosamine (GlcNAc) and N-acetylgalactosamine (GalNAc) residues. (ac.ir)
  • A hexosaminidase with specificity for terminal non-reducing N-acetyl-D-galactosamine residues in N-acetyl-alpha-D-galactosaminides. (harvard.edu)
  • Studies of the isolated isozymes showed that β-hexosaminidase B was more stable to heat and more active at low pH than β-hexosaminidase A. Kinetic studies revealed that the A and B forms of β-hexosaminidase had essentially the same K m and V max for ρ-nitro-phenyl-N-acetyl-β-D-glucosaminide. (nebraska.edu)
  • and hexosaminidase S is a homodimer of alpha chains. (wikipedia.org)
  • Alpha subunits of b-hexosaminidase A: absence or defects of these results in Tay-Sachs disease (TSD) and its variants. (patient.info)
  • One is chitinolytic β- N -acetyl-D-hexosaminidase, which is specifically involved in chitin degradation during insect metamorphosis [ 2 - 6 ]. (ijbs.com)
  • The second is the N -glycan processing β- N -acetyl-D-hexosaminidase, which is a membrane-bound enzyme that is involved in the degradation of N -glycans [ 7 - 9 ]. (ijbs.com)
  • Our data suggest that the N-acetyl-hexosaminidase is involved in degradation of knee joint extracellular matrix and can be good indicator of the degree of that degradation. (osteoporoza.pl)
  • Standardization of leukocytes for Tay-Sachs hexosaminidase assay [Abstract]. (thefreedictionary.com)
  • lysosomal for Tay Sachs, hexosaminidase A, total leukocytes/molecular reflex. (nicklauschildrens.org)
  • The recommended testing strategy is to order NAGR / Hexosaminidase A and Total, Leukocytes/Molecular Reflex, which begins with enzyme analysis and when the percent of hexosaminidase A enzyme is low, reflexes to the molecular panel which includes the most common mutations observed in these high-risk populations and 2 common pseudodeficiency alleles. (testcatalog.org)
  • Tay-Sachs disease occurs when hexosaminidase A loses its ability to function. (wikipedia.org)
  • The prototype hexosaminidase A deficiency is Tay-Sachs disease, also known as the acute infantile variant. (archive.org)
  • A new juvenile hexosaminidase deficiency disease presenting as cerebellar ataxia. (semanticscholar.org)
  • To assist in diagnosing Tay-Sachs disease by identifying a hexosaminidase enzyme deficiency. (unboundmedicine.com)
  • Tay-Sachs disease is an autosomal recessive condition resulting from 2 mutations in HEXA , which encodes for the alpha subunit of hexosaminidase. (testcatalog.org)
  • Menendez-Helman R J, Sanjurjo C, Miranda P V. Seminal plasma hexosaminidase in patients with normal and abnormal spermograms. (ac.ir)
  • Studies on the glycosidases of semen: purification & properties of two hexosaminidases of bull seminal plasma. (bvsalud.org)
  • Population-based Tay-Sachs screening among Ashkenazi Jewish young adults in the 21st century: Hexosaminidase A enzyme assay is essential for accura. (cdc.gov)
  • 17 Hexosaminidase A (HEXA) Kits ELISA de 10 fabricants sont disponibles sur www.anticorps-enligne.fr. (anticorps-enligne.fr)