Pompe disease is caused by a deficiency of a critical enzyme in the body called acid alpha glucosidase (GAA). Normally, GAA is used by the bodys cells to break down glycogen (a stored form of sugar) within specialized structures called lysosomes. In infants with severe cases of Pompe disease (called Classical Infantile Pompe disease), an excessive amount of glycogen accumulates and is stored in various tissues, especially heart, skeletal muscle, and liver, which prevents their normal function. This study being conducted to evaluate the safety and effectiveness of recombinant human acid alpha-glucosidase (rhGAA) as a potential enzyme replacement therapy for Pompe disease. Patients diagnosed with Classical Infantile Pompe disease who have a small, but inactive, amount of natural GAA enzyme present in their bodies (called Cross-Reacting Immunologic Material-Positive or CRIM (+) patients), will be studied ...
Under basic reaction conditions pyridinium or isoquinolinium N-ylides (C=5H=5N^+CH=2YRr^-, Y: CO=2R, CN, PhC0) reacted readily with 4-ethoxyl1-1,1,1-trifluorobut -3-en-2-one to give the corresponding 1-trifluoroacetyl substituted indolizines or pyrrolo-[1,2-a] isoquinolines. The molecular structure of 1-trifluoromethyl-3-methoxyl-pyrrolo[1,2-a]isoquinoline is presented ...
Glycogen storage disease type II, also called Pompe disease, is an autosomal recessive metabolic disorder which damages muscle and nerve cells throughout the body. It is caused by an accumulation of glycogen in the lysosome due to deficiency of the lysosomal acid alpha-glucosidase enzyme. It is the only glycogen storage disease with a defect in lysosomal metabolism, and the first glycogen storage disease to be identified, in 1932 by the Dutch pathologist J. C. Pompe. The build-up of glycogen causes progressive muscle weakness (myopathy) throughout the body and affects various body tissues, particularly in the heart, skeletal muscles, liver and the nervous system. The infantile form usually comes to medical attention within the first few months of life. The usual presenting features are cardiomegaly (92%), hypotonia (88%), cardiomyopathy (88%), respiratory distress (78%), muscle weakness (63%), feeding difficulties (57%) and failure to thrive (50%). The main clinical findings include floppy baby ...
Looking for online definition of Acid beta-glucosidase deficiency in the Medical Dictionary? Acid beta-glucosidase deficiency explanation free. What is Acid beta-glucosidase deficiency? Meaning of Acid beta-glucosidase deficiency medical term. What does Acid beta-glucosidase deficiency mean?
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BioAssay record AID 593427 submitted by ChEMBL: Inhibition of sweet almond beta-glucosidase after 10 mins using 4-nitrophenylbeta-D-glucopyranoside as a substrate by Lineweaver-Burk and double reciprocal analysis.
Polycystic liver disease (PLD) usually describes the presence of multiple cysts scattered throughout normal liver tissue, in association with polycystic kidney disease. Associations with PRKCSH and SEC63 have been described. Polycystic liver disease comes in two forms as autosomal dominant polycystic kidney disease (with kidney cysts) and autosomal dominant polycystic liver disease (liver cysts only). Online Mendelian Inheritance in Man (OMIM) 174050 Everson, Gregory T. "Polycystic Liver Disease". Gastroenterology & Hepatology. 4 (3): 179-181. ISSN 1554-7914. Retrieved 18 December 2017. http://www.pkdcure.org/site/PageServer?pagename=pkdabt_patientsartic6 http://www.hdcn.com/symp/01pkd/per/per1.htm (requires free account, register at http://www.hdcn.com/reg.htm) http://www.pkdiet.com/pld.php http://www.polycysticliverdisease.com/html/about_pld.html http://polycysticliverdisease.weebly.com ...
How is Nojirimycin 6-Phosphate abbreviated? N6P stands for Nojirimycin 6-Phosphate. N6P is defined as Nojirimycin 6-Phosphate very rarely.
A genomic library of Pseudomonas fluorescens subsp. cellulosa DNA constructed in pUC18 and expressed in Escherichia coli was screened for recombinants expressing 4-methylumbelliferyl beta-D-glucoside hydrolysing activity (MUGase). A single MUGase-positive clone was isolated. The MUGase hydrolysed cellobiose, cellotriose, cellotetraose, cellopentaose and cellohexaose to glucose, by sequentially cleaving glucose residues from the non-reducing end of the cello-oligosaccharides. The Km values for cellobiose and cellohexaose hydrolysis were 1.2 mM and 28 microM respectively. The enzyme exhibited no activity against soluble or insoluble cellulose, xylan and xylobiose. Thus the MUGase is classified as a 1,4-beta-D-glucan glucohydrolase (EC 3.2.1.74) and is designated 1,4-beta-D-glucan glucohydrolase D (CELD). When expressed by E. coli, CELD was located in the cell-envelope fraction; a significant proportion of the native enzyme was also associated with the cell envelope when synthesized by its ...
An autosomal recessively inherited glycogen storage disease caused by glucan 1,4-Alpa-Glucosidase Definciency. Large amounts of glycogen accumulate in the lysomes of skeletal muscle ( muscle, skeletal); heart; liver; spinal cord; and brain.
Rationale: Polycystic liver disease (PLD) is a rare disorder characterized by ,20 fluid-filled hepatic cysts. Polycystic livers are present in the combination with renal cysts as a manifestation of autosomal dominant polycystic kidney disease (ADPKD), or isolated in the absence of renal cysts as autosomal dominant polycystic liver disease (ADPLD or PCLD). PLD patients are confronted with symptoms caused by the mass effect of their polycystic liver every day for the rest of their life. There is no standard therapeutic option for symptomatic PLD patients. Current options are fairly invasive or their efficacy is only moderate.. Preliminary data in our research lab have shown that ursodeoxycholic acid (UDCA) inhibited the proliferation of polycystic human cholangiocytes in vitro through the normalization of the intracellular calcium levels in cystic cholangiocytes. The investigators also found that daily oral administration of UDCA for 5 months to PCK rats, an animal model of ARPKD that ...
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Glycogenosis type II is an inherited lysosomal storage disorder caused by acid α-glucosidase deficiency. The disorder is inbred in Brahman cattle, and the incidence of carriers in Australian herds averages 15%. Affected animals are lethargic and die typically in the eighth or ninth month after birth, A complete lack of acid α-glucosidase synthesis was demonstrated in cultured fibroblasts and muscle tissue of affected animals. Moreover, the tissue was found to be devoid of acid α-glucosidase mRNA. Gross abnormalities of the acid α-glucosidase gene itself were not detected by Southern blot analysis. These results suggest Brahman glycogenosis type II to be caused by a point mutation or a micro deletion/insertion in the acid α-glucosidase gene ...
The nucleotide sequence of the celY gene coding for the thermostable exo-1,4-β-glucanase Avicelase II of Clostridium stercorarium was determined. The gene consists of an ORF of 2742 bp which encodes a preprotein of 914 amino acids with a molecular mass of 103 kDa. The signal-peptide cleavage site was identified by comparison with the N-terminal amino acid sequence of Avicelase II purified from C. stercorarium. The celY gene is located in close vicinity to the celZ gene coding for the endo-1,4-β-glucanase Avicelase I. The CelY-encoding sequence was isolated from genomic DNA of C. stercorarium with the PCR technique. The recombinant enzyme produced in Escherichia coli as a LacZ'-CelY fusion protein could be purified using a simple two-step procedure. The properties of CelY proved to be consistent with those of Avicelase II purified from C. stercorarium. Sequence comparison revealed that CelY consists of an N-terminal catalytic domain flanked by a domain of 95 amino acids with unknown function
Prunasin is a cyanogenic glycoside related to amygdalin. Chemically, it is the glucoside of (R)-mandelonitrile. Prunasin is found in species in the genus Prunus such as Prunus japonica or P. maximowiczii and in bitter almonds. It is also found in leaves and stems of Olinia ventosa, O. radiata, O. emarginata and O. rochetiana and in Acacia greggii. It is also found in dandelion coffee, a coffee substitute. Sambunigrin, a diastereomer of prunasin derived from (S)-mandelonitrile instead of it the (R)-isomer, has been isolated from leaves of the elder tree (Sambucus nigra) Prunasin is hydrolyzed to produce hydrogen cyanide. Plants containing prunasin may therefore be toxic to animals, particularly ruminants. Prunasin beta-glucosidase is an enzyme that uses (R)-prunasin and H2O to produce D-glucose and mandelonitrile. Amygdalin beta-glucosidase is an enzyme that uses (R)-amygdalin and H2O to produce (R)-prunasin and D-glucose. Sanchez-Perez, R.; Belmonte, F. S.; Borch, J.; Dicenta, F.; Møller, B. ...
Autosomal dominant polycystic liver disease results from mutations in PRKCSH or SEC63. The respective gene products, glucosidase IIb and SEC63p, function in protein translocation and quality control pathways in the endoplasmic reticulum. Here we show that glucosidase IIb and Sec63p are required in m …
Glucosidases are glycoside hydrolase enzymes categorized under the EC number 3.2.1. α-glucosidases are enzymes involved in breaking down complex carbohydrates such as starch and glycogen into their monomers They catalyze the cleavage of individual glucosyl residues from various glycoconjugates including alpha- or beta-linked polymers of glucose.This enzyme convert complex sugars into the simpler one. Different sources include different members in this class. Members marked with a "#" are considered by MeSH to be glucosidases. They are targeted by alpha-glucosidase inhibitors such as acarbose and miglitol to control diabetes mellitus type 2. DNA glycosylases Mucopolysaccharidoses Glucosidases at the US National Library of Medicine Medical Subject Headings (MeSH) Molecular and Cellular Biology ...
... (AGIs) are a class of drugs used to treat type 2 diabetes. The key benefit of AGIs is that they do not cause hypoglycemia (low blood sugar) or weight gain and are taken orally. These drugs help lower blood sugar levels in the body by blocking the breakdown of starchy foods like bread and potatoes in the intestine and thus delaying the absorption of carbohydrates, according to the American Diabetes Association They also slow down the breakdown of some sugars, like table sugar. Because they work to slow digestion, AGIs are taken at the start of a meal. The alpha-glucosidase inhibitors available today (with brand names in parenthesis) are: acarbose (Precose) miglitol (Glyset) An ADA published article states that
K12316 GAA; lysosomal alpha-glucosidase [EC:3.2.1.20] K00963 UGP2; UTP--glucose-1-phosphate uridylyltransferase [EC:2.7.7.9] K00963 UGP2; UTP--glucose-1-phosphate uridylyltransferase [EC:2.7.7.9] K01513 ENPP1_3; ectonucleotide pyrophosphatase/phosphodiesterase family member 1/3 [EC:3.1.4.1 3.6.1.9] K05349 bglX; beta-glucosidase [EC:3.2.1.21] K05349 bglX; beta-glucosidase [EC:3.2.1.21] K05349 bglX; beta-glucosidase [EC:3.2.1.21] K05349 bglX; beta-glucosidase [EC:3.2.1.21] K05349 bglX; beta-glucosidase [EC:3.2.1.21] K01225 CBH1; cellulose 1,4-beta-cellobiosidase [EC:3.2.1.91] K01225 CBH1; cellulose 1,4-beta-cellobiosidase [EC:3.2.1.91] K00693 GYS; glycogen synthase [EC:2.4.1.11] K00750 GYG1; glycogenin [EC:2.4.1.186] K00750 GYG1; glycogenin [EC:2.4.1.186] K00750 GYG1; glycogenin [EC:2.4.1.186] K00750 GYG1; glycogenin [EC:2.4.1.186] K00700 GBE1; 1,4-alpha-glucan branching enzyme [EC:2.4.1.18] K00688 PYG; glycogen phosphorylase [EC:2.4.1.1] K01196 AGL; glycogen debranching enzyme [EC:2.4.1.25 ...
THONG, WONG KUM (2015) A generic assay for whole-genome amplification and deep sequencing of enterovirus A71. Journal of Virological Methods. pp. 30-36. THONG, WONG KUM (2015) Enterovirus 71 neuropathogenesis and its impact on other neurotropic enteroviruses. Brain Pathology, 25. pp. 614-624. THONG, WONG KUM (2014) Late-onset glycogen storage disease Type II (Pompes Disease) with a novel mutation: A Malaysian perspective. Case reports in Neurological Medicine, 2014. doi. 10.115/2014/926510. THONG, WONG KUM (2013) Protection against henipavirus infection using recombinant AAV vector vaccines. pp. 469-478. THONG, WONG KUM (2013) Transgenic mouse model for the study of enterovirus 71 neuropathogenesis. Proceedings of the National Academy of Sciences of the United States of America, 110. pp. 14753-14758. THONG, WONG KUM (2012) Clinical and pathological manifestations of human Henipavirus infection. THONG, WONG KUM (2012) Immunization with recombinant enterovirus 71 viral capsid protein 1 fragment ...
Haemolysin BL (HBL) is a Bacillus cereus toxin composed of a binding component, B, and two lytic components, L1 and L2. HBL is also the enterotoxin responsible for the diarrhoeal food poisoning syndrome caused by several strains of B. cereus. The three genes encoding the HBL components constitute an operon and are transcribed from a promoter 608 bp upstream of the hblC translational start site. The first gene of the hbl operon, hblC, in the B. cereus type strain, ATCC 14579, was inactivated in this study. Inactivation of hblC strongly reduced both the enterotoxigenic activity of B. cereus ATCC 14579 and the haemolytic activity against sheep erythrocytes, while maintaining full haemolytic activity against human erythrocytes.
TY - JOUR. T1 - 3′-methylphosphonate-modified oligo-2′-O-methylribonucleotides and their Tat peptide conjugates. T2 - Uptake and stability in mouse fibroblasts in culture. AU - Prater, Chrissy E.. AU - Miller, Paul S.. PY - 2004/5/1. Y1 - 2004/5/1. N2 - Antisense oligo-2′-O-methylribonucleotides and their methylphosphonate derivatives show high binding affinities for their complementary targets under essentially physiological conditions. Additionally, the methylphosphonate linkage is resistant to nuclease hydrolysis. Here we show that a single methylphosphonate internucleotide linkage at the 3′-end of an oligo-2′-O-methylribonucleotide is sufficient to prevent degradation by the 3′-exonuclease activity found in mammalian serum. Complexes formed between a cationic lipid, Oligofectamine, and 5′-[32P]-labeled methylphosphonate modified oligo2′-O-methylribonucleotides are taken up by mouse L929 fibroblasts in culture. The extent of uptake appears to be dependent upon the sequence of ...
Glycoside hydrolases (O-Glycosyl hydrolases) EC 3.2.1. are a widespread group of enzymes that hydrolyse the glycosidic bond between two or more carbohydrates, or between a carbohydrate and a non-carbohydrate moiety. A classification system for glycosyl hydrolases, based on sequence similarity, has led to the definition of numerous different families. This classification is available on the CAZy (CArbohydrate-Active EnZymes) web site. Because the fold of proteins is better conserved than their sequences, some of the families can be grouped in clans. As of October 2011, CAZy includes 128 families of glycosyl hydrolases and 14 clans. Glycoside hydrolase family 1 Glycoside hydrolase family 2 Glycoside hydrolase family 3 Glycoside hydrolase family 4 Glycoside hydrolase family 5 Glycoside hydrolase family 6 Glycoside hydrolase family 7 Glycoside hydrolase family 8 Glycoside hydrolase family 9 Glycoside hydrolase family 10 Glycoside hydrolase family 11 Glycoside hydrolase family 12 Glycoside ...
The subject of the invention is benzofuran, benzothiophene, indole or indolizine compounds of general formula: ##STR1## in which: Het represents one of the groups: ##STR2## in which T,T and Treprese
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Glycogen storage disease type Ia (GSDIa) is an inherited disorder of glucose metabolism, due to the selective deficiency of the hepatic enzyme glucose-6-phosphatase. Clinical manifestations include severe hypoglycaemia three to four hours post-prandially, increased production of lactic acid, triglycerides and uric acid, hepatic glycogen storage disease with development of multiple adenomas and kidney disease with proteinuria. Liver transplantation is frequently performed in order to achieve metabolic control and when malignant transformation of adenomas is suspected. Long term outcome following transplantation is good, but immunosuppressive therapy can worsen the progression of associated kidney disease. Hepatocyte transplantation could be considered as a less invasive procedure in such patients. Our experience with hepatocyte transplantation in a 47 year-old woman affected by glycogen storage disease type Ia and suffering of severe fasting hypoglycaemia indicates that the procedure can ...
Patients with Niemann-Pick disease type C1 (NPC1), a lysosomal lipid storage disorder that causes neurodegeneration and liver damage, can present with IBD, but neither the significance nor the functional mechanism of this association is clear. We studied bacterial handling and antibacterial autophagy in patients with NPC1.We characterised intestinal inflammation in 14 patients with NPC1 who developed IBD. We investigated bacterial handling and cytokine production of NPC1 monocytes or macrophages in vitro and compared NPC1-associated functional defects to those caused by IBD-associated nucleotide-binding oligomerization domain-containing protein 2 (NOD2) variants or mutations in X-linked inhibitor of apoptosis (XIAP).Patients with the lysosomal lipid storage disorder NPC1 have increased susceptibility to early-onset fistulising colitis with granuloma formation, reminiscent of Crohns disease (CD). Mutations in NPC1 cause impaired autophagy due to defective autophagosome function that abolishes NOD2
Sucrose intolerance, also called sucrase-isomaltase deficiency, congenital sucrase-isomaltase deficiency (CSID), or genetic sucrase-isomaltase deficiency (GSID), is the condition in which sucrase-isomaltase, an enzyme needed for proper metabolism of sucrose (sugar) and starch (i.e., grains and rice), is not produced or the enzyme produced is either partially functional or non-functional in the small intestine. All GSID patients lack fully functional sucrase, while the isomaltase activity can vary from minimal functionality to almost normal activity. The presence of residual isomaltase activity may explain why some GSID patients are better able to tolerate starch in their diet than others with GSID. The highest prevalence rates are seen in the Inuit populations of Greenland (5-10%), Alaska (3-7%) and Canada (about 3%). European descent prevalence ranges from 0.2% to 0.05%. There is a lower prevalence reported in African Americans and Hispanics compared to Caucasians. Abdominal cramps and bloating ...
Glycogenosis type VII: …a deficiency in muscle phosphorylase; type VII, a deficiency in phosphofructokinase; type VIII, a deficiency in phosphohexoisomerase; and type X, a deficiency in phosphorylasekinase-are diseases that are characterized by weakness, muscle cramps, and sometimes myoglobinuria.
K12316 GAA; lysosomal alpha-glucosidase [EC:3.2.1.20] K12316 GAA; lysosomal alpha-glucosidase [EC:3.2.1.20] K00963 UGP2; UTP--glucose-1-phosphate uridylyltransferase [EC:2.7.7.9] K00963 UGP2; UTP--glucose-1-phosphate uridylyltransferase [EC:2.7.7.9] K05349 bglX; beta-glucosidase [EC:3.2.1.21] K00694 bcsA; cellulose synthase (UDP-forming) [EC:2.4.1.12] K01179 E3.2.1.4; endoglucanase [EC:3.2.1.4] K01179 E3.2.1.4; endoglucanase [EC:3.2.1.4] K01179 E3.2.1.4; endoglucanase [EC:3.2.1.4] K01179 E3.2.1.4; endoglucanase [EC:3.2.1.4] K01179 E3.2.1.4; endoglucanase [EC:3.2.1.4] K01179 E3.2.1.4; endoglucanase [EC:3.2.1.4] K01179 E3.2.1.4; endoglucanase [EC:3.2.1.4] K01179 E3.2.1.4; endoglucanase [EC:3.2.1.4] K01225 CBH1; cellulose 1,4-beta-cellobiosidase [EC:3.2.1.91] K01225 CBH1; cellulose 1,4-beta-cellobiosidase [EC:3.2.1.91] K18447 NUDX14; ADP-sugar diphosphatase [EC:3.6.1.21] K00693 GYS; glycogen synthase [EC:2.4.1.11] K00700 GBE1; 1,4-alpha-glucan branching enzyme [EC:2.4.1.18] K00688 PYG; glycogen ...
Niemann-Pick Disease Type D (Niemann-Pick Disease Type Nova Scotia): Read more about Symptoms, Diagnosis, Treatment, Complications, Causes and Prognosis.
K01187 malZ; alpha-glucosidase [EC:3.2.1.20] K00963 UGP2; UTP--glucose-1-phosphate uridylyltransferase [EC:2.7.7.9] K00963 UGP2; UTP--glucose-1-phosphate uridylyltransferase [EC:2.7.7.9] K05349 bglX; beta-glucosidase [EC:3.2.1.21] K05349 bglX; beta-glucosidase [EC:3.2.1.21] K05350 bglB; beta-glucosidase [EC:3.2.1.21] K01179 E3.2.1.4; endoglucanase [EC:3.2.1.4] K00703 glgA; starch synthase [EC:2.4.1.21] K00693 GYS; glycogen synthase [EC:2.4.1.11] K00700 GBE1; 1,4-alpha-glucan branching enzyme [EC:2.4.1.18] K00688 PYG; glycogen phosphorylase [EC:2.4.1.1] K01176 AMY; alpha-amylase [EC:3.2.1.1] K05343 treS; maltose alpha-D-glucosyltransferase / alpha-amylase [EC:5.4.99.16 3.2.1.1] K05343 treS; maltose alpha-D-glucosyltransferase / alpha-amylase [EC:5.4.99.16 3.2.1.1] K21574 susB; glucan 1,4-alpha-glucosidase [EC:3.2.1.3] K01182 IMA; oligo-1,6-glucosidase [EC:3.2.1.10] K00705 malQ; 4-alpha-glucanotransferase [EC:2.4.1.25] K16147 glgE; starch synthase (maltosyl-transferring) [EC:2.4.99.16] K00691 ...
A novel type of model substrates, i.e. immobilized p-aminophenyl-β-D-cellooligosaccharides, was developed and used in the study of exocellulases. The two major cellobiohydrolases from Trichoderma reesei, CBH I and CBH II were used as representative enzymes. p-Aminophenyl derivatives of cellobiose (PAPG₂), cellotriose (PAPG₃), and cellotetraose (PAPG₄) were synthesized from the reaction of p-nitrophenol and peracetylated glycosyl bromide of the corresponding cellooligosaccharides under the phase-transfer catalyzed conditions, followed by deacetylation and catalytic hydrogenation. p-Aminophenyl cellooligosaccharides were then tethered via their amino functional groups to N-hydroxy succinimide-activated agarose. The ability of CBH I and CBH II to associate with and catalyze the hydrolysis of reducing end tethered cellooligosaccharides was tested. CBH I catalyzed the hydrolysis of free PAPG₂ but CBH II did not. Both CBH I and CBH II reversibly bound, but did not hydrolyze, immobilized ...
Price: US$ 5,400.00. It is estimated that the output of GDL will increase but will not increase a lot. The reasons are listed below.. Firstly, the production cost of GDL will increase, so the price of GDL may keep at a high level, which will in turn restrict the application of GDL. Secondly, the demand for GDL from food industry will increase stably. On one hand, the application field of GDL is still very limited in China by 2012. And it is believed that this situation will not change fast in the coming years because many downstream industries are unfamiliar with GDL at present. On the other hand, as domestic bean curd industry, the largest user of GDL in China, is relatively mature, both the output of bean curd and the market share of lactone bean curd will increase slowly in the coming years.. More information can be found in the report "Production and Market of Glucono-delta-lactone in China" by Guangzhou CCM Information Science & Technology.. To order the report or ask for sample pages ...
This study focused on the synthesis and characterization of hydrazide ligands and their respective Pd(II) complexes and used high throughput screening to determine their α-glucosidase and carbonic anhydrase II enzyme inhibition activities. The physical, analytical (elemental analyses for C, H, N and Pd) and spectral (FT-IR, 1H NMR, 13C NMR, EI-mass) techniques utilized during characterization revealed the formation of square planar, neutral and 1:2 Pd(II)-hydrazide complexes with the general formula [PdL2Cl2]. In these Pd(II) complexes, the hydrazide ligands are monodentate; the terminal nitrogen is the donor atom. The uncoordinated hydrazide ligands were inactive against both α-glucosidase and carbonic anhydrase II enzymes; however, the respective Pd(II)-hydrazide complexes were approximately 300 times more potent α-glucosidase inhibitors than the standard compound, 1-deoxynojirimycin (DNJ). Some of the Pd(II) complexes also demonstrated potential carbonic anhydrase (CA) inhibition ...
Phenolic composition of virgin olive oil is determined by the enzymatic and/or chemical reactions that take place during olive fruit processing. Of these enzymes, β-glucosidase activity plays a key role in the transformation of the phenolic glycosides present in the olive fruit, generating different secoiridoid derivatives. The main goal of the present study was to characterize olive fruit β-glucosidase genes and enzymes responsible for the phenolic composition of virgin olive oil. To achieve that, we have isolated an olive β-glucosidase gene from cultivar Picual (OepGLU), expressed in Nicotiana benthamiana leaves and purified its corresponding recombinant enzyme. Western blot analysis showed that recombinant OepGLU protein is detected by an antibody raised against the purified native olive mesocarp β-glucosidase enzyme, and exhibits a deduced molecular mass of 65.0 kDa. The recombinant OepGLU enzyme showed activity on the main olive phenolic glycosides, with the highest levels toward oleuropein,
Clark, NE, Garman, SC. The 1.9 A structure of human alpha-N-acetylgalactosaminidase: The structural basis of Schindler and Kanzaki diseases. Journal of Molecular Biology. 2009, Oct 23;393(2):435-447. [PubMed]. Ishii S, Chang HH, Kawasaki K, Yasuda K, Wu HL, Garman SC, Fan JQ. Mutant alpha-galactosidase A enzymes identified in Fabry disease patients with residual enzyme activity: biochemical characterization and restoration of normal intracellular processing by 1-deoxygalactonojirimycin. Biochem J. 2007 Sep 1;406(2):285-95.[PubMed]. Hebert DN, Garman SC, Molinari M. (2005) "The glycan code of the endoplasmic reticulum: asparagine-linked carbohydrates as protein maturation and quality-control tags." Trends Cell Biol. Jul;15(7):364-70. Review.[PubMed]. Su HP, Garman SC, Allison TJ, Fogg C, Moss B, Garboczi DN. (2005) "The 1.51-Angstrom structure of the poxvirus L1 protein, a target of potent neutralizing antibodies." Proc Natl Acad Sci U S A. Mar 22;102(12):4240-5. Epub 2005 Mar 10. [PubMed]. Ries ...
β-glucosidase (EC 3.2.1.21); β-galactosidase (EC 3.2.1.23); β-mannosidase (EC 3.2.1.25); β-glucuronidase (EC 3.2.1.31); β-xylosidase (EC 3.2.1.37); β-D-fucosidase (EC 3.2.1.38); phlorizin hydrolase (EC 3.2.1.62); exo-β-1,4-glucanase (EC 3.2.1.74); 6-phospho-β-galactosidase (EC 3.2.1.85); 6-phospho-β-glucosidase (EC 3.2.1.86); strictosidine β-glucosidase (EC 3.2.1.105); lactase (EC 3.2.1.108); amygdalin β-glucosidase (EC 3.2.1.117); prunasin β-glucosidase (EC 3.2.1.118); vicianin hydrolase (EC 3.2.1.119); raucaffricine β-glucosidase (EC 3.2.1.125); thioglucosidase (EC 3.2.1.147); β-primeverosidase (EC 3.2.1.149); isoflavonoid 7-O-β-apiosyl-β-glucosidase (EC 3.2.1.161); ABA-specific β-glucosidase (EC 3.2.1.175); DIMBOA β-glucosidase (EC 3.2.1.182); β-glycosidase (EC 3.2.1.-); hydroxyisourate hydrolase (EC 3.-.-.- ...
Membrane proteins of the intestinal brush border were labelled in vivo by intraluminal injection of diazotised [125I]iodosulfanilic acid, a highly polar molecule. Sodium dodecyl sulfate polyacrylamide gel electrophoresis of brush border membranes labelled in this manner showed 20 protein bands, 11 of which contained significant radioactivity. The most heavily labelled proteins had molecular weights greater than 150000, indicating that they were the most exposed to the intestinal lumen. Little radioactivity was detected in proteins with molecular weights of less than 94000. The majority of these smaller proteins were likely to have been brush border core proteins. The evidence that diazotised [125I]iodosulfanilic acid bound primarily to brush border membrane proteins when administered in this way, was: (a) the specific activity of brush border proteins was up to 3-fold greater than that of total cell particulate proteins (pelleted by 27000 x g from mucosal homogenates); (b) principal peaks in the gel
Autosomal dominant polycystic kidney disease (ADPKD) is a life-threatening monogenic disease with a prevalence of 1 in 400-1000 livebirths. ADPKD is caused by mutations to polycystic kidney disease 1 gene (PKD1) (approximately 85% of cases) or polycystic kidney disease 2 gene (PKD2) (the remaining 15%) gene, encoding polycystin-1 (PC1) and polycystin-2 (PC2), respectively. PC1 is a putative cell-surface, receptor-like protein with yet to-be-identified ligand(s), and PC2 a channel protein with a high conductance to Ca2+.. Polycystic liver disease (PLD) is the most common extra-renal manifestation in ADPKD, present in , 90% of ADPKD patients by age 30. Liver cysts in ADPKD originate from biliary micro-hamartoma or focal proliferations of biliary ductules and from peribiliary glands. Excessive proliferation of biliary epithelial cells, combined with neovascularization, altered cell-extracellular matrix (ECM) interaction/ECM remodeling and cAMP-mediated fluid secretion, is required for the ...
Deoxynojirimycin (DNJ) is the archetypal iminosugar, in which the configuration of the hydroxyl groups in the piperidine ring truly mimic those of d-glucopyranose; DNJ and derivatives have beneficial effects as therapeutic agents, such as anti-diabetic and antiviral agents, and pharmacological chaperones for genetic disorders, because they have been shown to inhibit α-glucosidases from various sources. However, attempts to design a better molecule based solely on structural similarity cannot produce selectivity between α-glucosidases that are localized in multiple organs and tissues, because the differences of each sugar-recognition site are very subtle. In this study, we provide the first example of a design strategy for selective lysosomal acid α-glucosidase (GAA) inhibitors focusing on the alkyl chain storage site. Our design of α-1-C-heptyl-1,4-dideoxy-1,4-imino-l-arabinitol (LAB) produced a potent inhibitor of the GAA, with an IC50 value of 0.44 µM. It displayed a remarkable ...
BioMed Research International is a peer-reviewed, Open Access journal that publishes original research articles, review articles, and clinical studies covering a wide range of subjects in life sciences and medicine. The journal is divided into 55 subject areas.
Antimicrobial chemicals are widely applied to clean and disinfect food-contacting surfaces. However, the cellular response of bacteria to various disinfectants is unclear. In this study, the physiological and genome-wide transcriptional responses of Bacillus cereus ATCC 14579 exposed to four different disinfectants (benzalkonium chloride, sodium hypochlorite, hydrogen peroxide, and peracetic acid) were analyzed. For each disinfectant, concentrations leading to the attenuation of growth, growth arrest, and cell death were determined. The transcriptome analysis revealed that B. cereus, upon exposure to the selected concentrations of disinfectants, induced common and specific responses. Notably, the common response included genes involved in the general and oxidative stress responses. Exposure to benzalkonium chloride, a disinfectant known to induce membrane damage, specifically induced genes involved in fatty acid metabolism. Membrane damage induced by benzalkonium chloride was confirmed by ...
Lactococcus lactis is being developed as a vaccine delivery system as it bears no threat to animal and human health. It has been used for centuries in the fermentation of foods and is generally recognised as safe. However, a safety factor pertaining to the type of selectable marker present on the vector system poses to be of concern. Chances of the transfer of antibiotic resistance genes, usually employed by vectors as selectable markers, into the natural environment become a possible risk. Therefore, there is a need for the development of ideal vectors without such selectable markers (Dertzbaugh, 1998). The activity of the xylanase gene of Bacillus coagulans ST -6 can be detected on Remazol Brilliant Blue-Xylan (RBB-Xylan) as a clear halo zone against a dark blue background. This characteristic allows xylanase to be used as a selectable chromogenic marker on any vector system. On the other hand, the matrix or membrane (M) protein of Newcastle disease virus (NDV) strain AF 2240 can be useful as ...
Looking for online definition of Niemann-Pick disease, type C1 in the Medical Dictionary? Niemann-Pick disease, type C1 explanation free. What is Niemann-Pick disease, type C1? Meaning of Niemann-Pick disease, type C1 medical term. What does Niemann-Pick disease, type C1 mean?
During constant work-rate exercise above the lactic acidosis threshold, oxygen consumption fails to plateau by 3 minutes, but continues to rise slowly. This slow component correlates closely with the rise in lactate in normal subjects. We investigated if oxygen consumption during constant work-rate exercise could rise after 3 minutes in the absence of a rise in lactate. We studied five patients with McArdles disease, one patient with phosphofructokinase deficiency and six normal subjects. Subjects performed two 6-minute duration constant work-rate exercise tests at 40 and 70% of peak oxygen consumption. During low-intensity exercise, oxygen consumption reached steady state by 3 minutes in both groups. Lactate rose slightly in control subjects but not in patients. During high-intensity exercise, oxygen consumption rose from the third to the sixth minute by 144 (21-607) ml/minute (median and range) in control subjects and by 142 (73-306) ml/minute in patients (p = not significant, Mann-Whitney U ...
Glucosidase II is an endoplasmic-reticulum-localized enzyme that cleaves the two internally alpha-1,3-linked glucosyl residues of the oligosaccharide Glc alpha 1----2Glc alpha 1----3Glc alpha 1----3Man5-9GlcNAc2 during the biosynthesis of asparagine-linked glycoproteins. We have purified this enzyme to homogeneity from the lactating bovine mammary gland. The enzyme is a high-mannose-type asparagine-linked glycoprotein with a molecular mass of approx. 290 kDa. Upon SDS/polyacrylamide-gel electrophoresis under reducing conditions, the purified enzyme shows two subunits of 62 and 64 kDa, both of which are glycosylated. The pH optimum is between 6.6 and 7.0. Specific polyclonal antibodies raised against the bovine mammary enzyme also recognize a similar antigen in heart, liver and the mammary gland of bovine, guinea pig, rat and mouse. These antibodies were used to develop a sensitive enzyme-linked immunosorbent assay for glucosidase II. ...
Previous studies [Büller, Montgomery, Sasak & Grand (1987) J. Biol. Chem. 262, 17206-17211] have demonstrated that lactase-phlorizin hydrolase is inserted into the microvillus membrane (MVM) as a large precursor of approx. 220 kDa, which then undergoes two proteolytic cleavage steps to become the 130 kDa mature MVM protein. In order to assess the role of glycosylation in intracellular transport, the processing of this enzyme has been studied in the presence of castanospermine, an inhibitor of N-linked oligosaccharide modification and subsequent treatment with two endoglycosidases, endo-beta-N-acetyl-glucosaminidase (endo-H) and peptide:N-glycosidase-F (N-glycanase). We now show that the intracellular precursor (205 kDa) undergoes carbohydrate processing (220 kDa) and transport to the MVM where its further proteolytic cleavage is as described. Treatment of the intracellular 205 kDa precursor with either endo-H which cleaves only high-mannose N-linked oligosaccharides, or with N-glycanase, which ...
Gaucher disease is a lysosomal storage disorder resulting from a deficiency in the key enzyme beta-glucocerebrosidase (GCase). The enzyme deficiency is caused by genetic mutations, which can result in the production of misfolded GCase. AT2101 is designed to act as a pharmacological chaperone by selectively binding to the misfolded GCase and helping it fold correctly, which may restore GCase activity.. This study is being conducted to test the safety of AT2101 in patients with type 1 Gaucher disease already receiving enzyme replacement therapy (ERT). The study will involve six visits over a period of 7 weeks and will evaluate the safety of AT2101. ...
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