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  • lysosomal storage disease
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  • Lysosomal membrane
    ¶óÀ̼Ҽظ·(¡­Ø­)
  • Glycolytic enzymes
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  • Intestinal mucosa, digestive enzymes
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  • hellp(hemolysis, elevated liver enzymes, low platelet),heliminths
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  • enzymes
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  • enzymes
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  • hellp(hemolysis, elevated liver enzymes, low platelet),heliminths
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  • iron porphyrin protein enzymes
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  • restriction enzymes
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  • inflammation,lysosomal constituents
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  • lysosomal acid phosphatase
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  • lysosomal granule
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  • lysosomal storage diseaes
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  • lysosomal storage disease
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  • lysosomal disease
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  • lysosomal storage disease
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HELLP hemolysis, elevated liver enzymes, and low platelet count [syndrome]
LAL left axillary line; Limulus amebocyte lysate; low air loss; lysosomal acid lipase
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LAL Lysosomal acid lipase
LAP Lysosomal acid phosphatase
LSD Lysosomal storage diseases
ML mitochondrial lysosomal
CYPs Cytochrome P450 enzymes
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inborn lysosomal disease Inherited disorder of one or more degradative enzymes normally located in lysosomes leading to accumulation (storage) of abnormal quantities of a substance, such as a glycosaminoglycan as in Hurler's syndrome or a lipopolysaccharide as in Gaucher's disease.
(05 Mar 2000)
UDP-N-acetylglucosamine-lysosomal-enzyme-N-acetylglucosaminephosphotransferase <enzyme> Fibroblasts from patients with i-cell (mucolipidosis II) and pseudo-hurler polydystrophy (mucolipidosis III) are deficient in above enzyme; for n-acetylglucosamine transferred to dolichyl phosphate see EC 2.7.8.15
Registry number: EC 2.7.8.17
Synonym: udpgnac gp gnac phosphotransferase, udpgnac phosphotransferase, uridine 5'-diphosphate-n-acetylglucosamine glycoprotein n-acetylglucosaminylphosphotransferase, n-acetylglucosamine-1-phosphotransferase, n-agapt, udp-n-acetylglucosamine-lysosomal glycoprotein n-acetylglucosaminylphosphotransferase, udp-acetylglucosamine-glycoprotein n-acetylglucosamine-1-phosphotransferase
(26 Jun 1999)
UDP-N-acetylglucosamine:lysosomal enzyme N-acetylglucosaminyl-1-phosphotransferase <enzyme> An enzyme that participates in the posttranslational modification of a number of lysosomal proteins; a deficiency or defect in this enzyme results in two forms of mucolipidoses, I-cell disease, and pseudo-Hurler polydystrophy.
(05 Mar 2000)
lysosomal disease A disease due to inadequate functioning of a lysosomal enzyme; most such disease's are associated with a storage disease.
(05 Mar 2000)
lysosomal diseases Diseases (also called storage diseases) in which a deficiency of a particular lysosomal enzyme leads to accumulation of the undigested substrate for that enzyme within cells. Not immediately fatal, but within a few years lead to serious neurological and skeletal disorders and eventually to death.
See: the following conditions: Hurler sundrome, Hunter syndrome, San Fillipo, Gaucher's disease, Niemann-Pick, Pompe's disease, Tay Sachs disease.
(18 Nov 1997)
lysosomal enzyme <biochemistry> A range of degradative enzymes, most of which operate best at acid pH. The best known marker enzymes are acid phosphatase and glucuronidase, but many others are known.
(18 Nov 1997)
lysosomal storage diseases Inborn errors of metabolism characterised by defects in specific lysosomal hydrolases and resulting in intracellular accumulation of unmetabolised substrates.
(12 Dec 1998)
complement activating enzymes <enzyme> Enzymes present in the complement system which activate one or more components in the system.
Registry number: EC 3.-
(12 Dec 1998)
hydrolyzing enzymes <enzyme> Registry number: EC 3.
(12 Dec 1998)
splitting enzymes Enzyme's that, like aldolases, catalyze the conversion of a molecule into two smaller molecules without the addition or subtraction of any atoms.
(05 Mar 2000)
deamidizing enzymes <enzyme> Registry number: EC 3.5.
(12 Dec 1998)
deaminating enzymes Enzymes catalyzing simple hydrolysis of C-NH2 bonds of purines, pyrimidines, and pterins, usually named in terms of the substrate, e.g., guanine deaminases, adenosine deaminases, AMP deaminases, pterin deaminases and thus producing ammonia; not generally used for deamination of noncyclic amides. Deaminases are distinguished from ammonia-lyases (EC group 4.3.1) in that the latter produce an unsaturation at the point of NH3 removal.
Synonym: deaminating enzymes.
(05 Mar 2000)
debranching enzymes Enzyme's that bring about destruction of branches in glycogen; formerly considered to be one enzyme, now known to be a mixture of transferases (4-alpha-d-glucanotransferase) and hydrolases (amylo-1,6-glucosidase).
Synonym: debranching factors.
(05 Mar 2000)
digestive enzymes Enzymes that are utilised in the digestive system, enzymes that are hydrolases of macromolecules (e.g., amylases, proteinases).
(05 Mar 2000)
DNA restriction enzymes <enzyme> Enzymes that are part of the restriction-modification systems. They catalyze the endonucleolytic cleavage of DNA sequences which lack the species-specific methylation pattern in the host cell's DNA. Cleavage yields random or specific double-stranded fragments with terminal 5'-phosphates. The function of restriction enzymes is to destroy any foreign DNA that invades the host cell. most have been studied in bacterial systems, but a few have been found in eukaryotic organisms. They are also used as tools for the systematic dissection and mapping of chromosomes, in the determination of base sequences of dnas, and have made it possible to splice and recombine genes from one organism into the genome of another.
Registry number: EC 3.1.21
(12 Dec 1998)
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