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Topic: Glucocerebroside

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	Gaucher's Disease
		Gaucher's disease is an inherited metabolic disorder in which harmful quantities of a fatty substance called glucocerebroside accumulate in the spleen, liver, lungs, bone marrow, and, in rare cases, the brain.
		Except for the brain, glucocerebroside arises mainly from the biodegradation of old red and white blood cells.
		In the brain, glucocerebroside arises from the turnover of complex lipids during brain development and the formation of the myelin sheath of nerves.
	healthlink.mcw.edu /article/921728377.html (370 words)

	What Causes Gaucher Disease? \| Caremark Health Resources
		Glucocerebroside is a fatty substance that is used as a building block to make certain cell membranes.
		In the brain, glucocerebroside comes from the processing of lipids during brain development and the formation of the myelin sheath (the fatty coating around each nerve fiber).
		Glucocerebroside is recycled when an enzyme, glucocerebrosidase, breaks it into glucose and a fat called ceramide.
	healthresources.caremark.com /topic/gauchercause (527 words)

	Cerezyme \| Online Glossary of Gaucher-Related Terms
		An example is glucocerebroside, which is composed of ceramide and glucose.
		Gaucher cells, as activated macrophages, were determined to be a source of chitotriosidase with the demonstration of massive secretion of the enzyme by cultured macrophages containing accumulated glucocerebroside.
		Gaucher cell: A cell containing excess glucocerebroside, which accumulates in the lysosomes because of a lack of the enzyme glucocerebrosidase.
	www.cerezyme.com /healthcare/resources/cz_hc_rsrc-glossary.asp (1724 words)

	Gaucher disease
		Deficiency of the enzyme ß-glucocerebrosidase leads to an accumulation of glucocerebroside in lysosomes of the monocyte-macrophage system.
		Deficiency of CGR causes the lysosomes to become congested with glucocerebroside.
		The mechanism of action is by inhibiting theformation of glucocerebroside.
	www.rwjobgyn.com /Atoz/encyclopedia/article/000564.asp (914 words)

	glucocerebroside - humpath.com (Site not responding. Last check: 2007-10-16)
		Glucocerebroside (also called glucosylceramide) is any of the cerebrosides in which the monosaccharide head group is glucose.
		They occur mostly in nonneuronal tissue and accumulate abnormally in Gaucher disease, where glucocerebrosidase is absent or nonfunctional.
		Gaucher disease is a defect in the degradation of glucocerebroside.
	www.humpath.com /spip.php?article9376 (45 words)

	Gaucher Disease: A guide for patients
		Glucocerebroside is a component of the cell membranes of red and white blood cells.
		In 1965, the American physician Roscoe O. Brady and coworkers demonstrated that the accumulation of glucocerebroside results from a deficiency of the enzyme glucocerebrosidase.
		Although a Gaucher carrier will have no symptoms of the disease, the odds are 50:50 that the "Gaucher gene" will be passed on to each of his or her children.
	www.gaucher.org.uk /living.htm (5450 words)

	Gaucher Disease: Unraveling A Mystery \| Caremark Health Resources
		Gaucher disease is marked by an overabundance of glucocerebroside -- a type of lipid, or fat molecule.
		Glucocerebroside is a key component of white blood cells.
		Liver biopsies were done a day before and a day after the enzyme was injected into two Gaucher patients to see whether there were any changes in the excess levels of glucocerebroside in their livers.
	healthresources.caremark.com /topic/audiogaucherscript (1921 words)

	Unknown Aspects of Gauchers Disease
		It appears that in Gauchers disease, the fatty material called glucocerebroside which is stored in the macrophages (scavenging cells) of the spleen, liver and bone marrow stimulates this response.
		In a normal individual, the total amount of glucocerebroside which accumulates in the combined organs would be 80 to 90 milligrams but in Gauchers disease it would be about 95 grams, still a small amount compared to the combined organs enlarged to a mass of 6.5 kilograms.
		There are other effects, probably toxic effects of the accumulated glucocerebroside in the brain cells as well as the macrophages in the brain.
	www.gaucher.org.uk /cox_ital.htm (1595 words)

	Gaucher's disease : Epilepsy.com/Professionals
		Gaucher's disease is an autosomal-recessive lipidosis caused by a deficiency of acid b-glucosidase.
		72 Because acid b-glucosidase hydrolyzes glucocerebroside in lysosomes to glucose and ceramide, Gaucher's disease is characterized by the lysosomal accumulation of glucocerebrosides (primarily glucosylceramide).
		Glucocerebroside accumulates in cells of the liver, spleen, lymph nodes, and bone marrow, leading to organomegaly, lymphadenopathy, and skeletal pain.
	professionals.epilepsy.com /page/inborn_gd.html (310 words)

	Characterization of a Quasicrystalline Phase in Codispersions of Phosphatidylethanolamine and Glucocerebroside ... (Site not responding. Last check: 2007-10-16)
		Characterization of a Quasicrystalline Phase in Codispersions of Phosphatidylethanolamine and Glucocerebroside
		There is convincing evidence, for example, that glucocerebroside, unlike galactocerebroside, is synthesized and located preferentially on the cytoplasmic surface of cell membranes (Coste et al., 1986).
		Of the aminophospholipids, phosphatidylethanolamines (PEs) are also known to be most abundant in the cytoplasmic leaflet of cell membranes (Fishman and Brady, 1976; Thompson and Tillack, 1985; Balasubramanian and Schroit, 2003; Daleke, 2003), although there is evidence that some PE is also present in the outer leaflet (Gurr and Harwood, 1991).
	www.findarticles.com /p/articles/mi_qa3938/is_200404/ai_n9388718 (749 words)

	Slashdoc - Gauchers Disease (Site not responding. Last check: 2007-10-16)
		If in fact the child inherits the disease he or she will experience problems with the liver, spleen, lungs, bone marrow, and in some cases the brain.
		The disease is caused by excessive amounts of a fatty substance called glucocerebroside.
		Glucocerebroside accumulates in the organs when there is a deficiency of the enzyme, glucocerebrosidase.
	www.slashdoc.com /documents/88110 (620 words)

	GBA - Genetics Home Reference
		Although the accumulation of glucocerebroside damages these organs, researchers are not certain how this buildup leads to the characteristic signs and symptoms of Gaucher disease.
		As a result, glucocerebroside builds up in macrophages in the body's organs, leading to the signs and symptoms of this disease.
		Gaucher-like disease - caused by mutations in the GBA gene
	ghr.nlm.nih.gov /gene=gba (1253 words)

	Neuronopathic Gaucher Disease - 2nd edition - Family factsheets - GOSH and ICH
		Without the enzyme, glucocerebroside accumulates in the body, mostly in the liver and spleen, and to a lesser extent in the bones, lungs and other organs.
		In all forms of Gaucher Disease, there is a build-up of glucocerebroside in the liver and spleen.
		Most of the glucocerebroside (we will call it GC for short) in our body comes from the membranes of blood cells that have reached the end of their natural lives and have to be broken down.
	www.gosh.nhs.uk /factsheets/families/F040187/index.html (1632 words)

	Glucocerebroside treatment ameliorates ConA hepatitis by inhibition of NKT lymphocytes -- Margalit et al. 289 (5): G917 ...
		Glucocerebroside treatment ameliorates ConA hepatitis by inhibition of NKT lymphocytes -- Margalit et al.
		The effect of glucocerebroside on NKT cells is not known.
		Incubation with glucocerebroside (GC) led to marked inhibition of NKT cell proliferation in the presence of dendritic cells (DC) but not in their absence.
	ajpgi.physiology.org /cgi/content/full/289/5/G917 (5305 words)

	Gaucher
		Glucocerebroside is therefore not broken down and accumulates in the lysosomes with the result that the macrophages will not function normally.
		The macrophages (then called Gaucher cells) contain the undigested glucocerebroside and they accumulate in the spleen, liver and bone marrow, as well as in other parts of the body – e.g.
		Inheriting a single mutated copy of the gene does not lead to symptoms of Gaucher Disease, since the body will be able to produce enough glucocerebrosidase to prevent accumulation of glucocerebroside.
	www.gaucher.dk /fakta-indexuk.asp (888 words)

	Gaucher Disease
		inherited storage diseases, and all result from the deficiency of an enzyme called glucocerebrosidase, which is necessary for the breakdown of a particular fatty substance, glucocerebroside.
		This fatty substance is normally present in very small amounts in all body cells, but in patients with Gaucher disease, glucocerebroside is not broken down as it should be and becomes abnormally stored, primarily in unique cells called Gaucher cells.
		The major disease manifestations are due to the progressive storage of glucocerebroside in Gaucher cells in the bone marrow, spleen and liver.
	www.ntsad.org /S02/S02gaucher.htm (1410 words)

	Gaucher Disease
		Gaucher disease - a rare, inherited disorder - is characterized by decreased levels of the enzyme glucocerebrosidase.
		This deficiency of glucocerebrosidase leads to the accumulation of the lipid glucocerebroside within the lysosomes of the monocyte/macrophage system.
		Lipid-engorged cells with eccentric nuclei, known as gaucher cells, constitute the primary defect in gaucher disease.
	www.genzyme.ca /thera/cz/ca_en_p_tp_thera-cz.asp (477 words)

	Gaucher Disease: A Rarity in Three Types
		The glucocerebrosidase enzyme functions to break down the compound glucocerebroside, a fatty compound which usually is stored in all cells of the body in very small amounts.
		In Gaucher patients, an excess of glucocerebroside builds up in the body, and is stored abnormally in lysosome, or storage cells (3).
		Macrophages which are enlarged and contain an abnormal buildup of glucocerebroside are known as Gaucher cells.
	serendip.brynmawr.edu /bb/neuro/neuro03/web1/rsinger.html (1001 words)

	Background on Type 2 Gauchers
		This enzyme deficiency results in the accumulation of a fatty substance (glucocerebroside) which is normally produced during the recycling of cells in the body but is then broken down by the enzyme.
		Instead, the glucocerebroside remains stored within the macrophages, preventing them from functioning normally.
		These cells are the hallmark of the disease and will be found in the bone marrow, spleen, liver and brain.
	members.optusnet.com.au /~mkgregory/gaucher/about.html (827 words)

	National Gaucher Foundation - Clinical Course of Gaucher Disease (Site not responding. Last check: 2007-10-16)
		The symptoms associated with Gaucher Disease result from the accumulation of a fatty substance, a lipid called glucocerebroside.
		This lipid is a byproduct of the normal recycling of red blood cells.
		The presence of glucocerebroside seems to also trigger the loss of minerals in the bones, causing the bones to weaken, and can interfere with the bone's blood supply, causing areas of bone-death, or "infarctions".
	www.gaucherdisease.org /gd_clinical_course.html (184 words)

	Knockdown of chimeric glucocerebrosidase by green fluorescent protein-directed small interfering RNA
		Gaucher disease is an autosomal recessive disorder characterized by a heterogeneous set of signs and symptoms caused by the defective lysosomal hydrolysis of glucocerebroside and related glucosphingolipids.
		This defective hydrolysis results from dysfunction of the enzyme glucocerebrosidase (GBA; glucosylceramidase, acid ß-glucosidase, EC 3.2.1.45) which acts as the penultimate step in conversion of glucocerebroside to glucose and ceramide (Beutler and Grabowski, 2001).
		Accumulated glucocerebroside is taken-up by macrophages and subsequently deposited in the spleen, liver, and bone marrow.
	funpecrp.com.br /gmr/year2004/vol2-3/gmr0100_full_text.htm (1503 words)

	Gaucher Disease - Type I (Non-Neuronopathic)
		Enzyme deficiency results in accumulation of un-degraded glycolipid substrates, particularly glucocerebroside, within the lysosomes of macrophages.
		Accumulation of Gaucher cells (enlarged cells containing excess glucocerebroside) in organs and tissues results in multi-systemic manifestations.
		In Gaucher disease, a deficiency of the enzyme glucocerebrosidase leads to the accumulation of the lipid glucocerebroside within the lysosomes of the monocyte-macrophage system.
	www.lysosomallearning.com /healthcare/about/lsd_hc_abt_gaucher1.asp (301 words)

	Gaucher Disease in Ashkenazic Jews
		In 1934, a complex, fatty substance called glucocerebroside was isolated from the swollen spleen of a Gaucher patient.
		In 1965, my mentor, Dr. Roscoe Brady and his co-workers proved that the accumulation of glucocerebroside is due to the hereditary deficiency of a single essential enzyme called glucocerebrosidase.
		The enzyme glucocerebrosidase is 1ocated within lysosomes, and is responsible for breaking down glucocerebroside which comes from membranes of senescent red and white blood cells.
	www.gauchersoutheast.com /articles/ashkenazic.html (2481 words)

	Gaucher's disease - MayoClinic.com (Site not responding. Last check: 2007-10-16)
		Gaucher's disease — pronounced go-SHAYZ — is a rare, inherited metabolic disorder in which excessive amounts of a fatty substance (lipid) called glucocerebroside accumulate in the spleen, liver, lungs and bone marrow.
		Gaucher's disease is caused by a deficiency of an enzyme called glucocerebrosidase, which breaks down glucocerebroside.
		This means that both parents must carry the gene defect in order to pass the disease to their children.
	www.mayoclinic.com /health/gauchers-disease/AN00840 (366 words)

	ENLmedical.com: Conditions And Concerns: Medical Encyclopedia: Gaucher disease
		The juvenile form of Gaucher disease occurs in all nationalities and is also inherited as an autosomal recessive disorder.
		With all forms, abnormal deposits of glucocerebroside occur in the liver, spleen, and bone marrow.
		In the chronic form of Gaucher, an enlarged spleen (splenomegaly) may be the first sign of disease.
	www.enlmedical.com /article/000564.htm (501 words)

	eMedicine - Gaucher Disease : Article Excerpt by: Zuzana Baranova, MD, PhD (Site not responding. Last check: 2007-10-16)
		Pathophysiology: Glucocerebroside contains equimolar concentrations of sphingosine, fatty acid, and glucose.
		In the CNS, glucocerebroside is produced in the course of ganglioside metabolism.
		The greatest concentrations of glucocerebroside levels were found in the occipital cortex, with lesser concentrations elsewhere in the brain.
	www.emedicine.com /derm/byname/gaucher-disease.htm (600 words)

	eMedicine - Gaucher Disease : Article by Zuzana Baranova, MD, PhD (Site not responding. Last check: 2007-10-16)
		Patients with GD, particularly those older than 50 years, are reported to be at an increased risk of multiple myeloma, possibly due to potential chronic stimulation of the immune system and lymphoproliferation associated with storage of glucocerebroside in tissue macrophages.
		The glucocerebroside concentration is increased 2- to 3-fold in the plasma and more than 10- to 1000-fold in the spleen, but high levels can also be detected in the liver and in bone marrow.
		These structures are similar to the twisted bilayers that are characteristic of glucocerebroside deposits.
	www.emedicine.com /derm/topic709.htm (4369 words)

	Lysosomal Storage Disorders: Inherited Disorders of Metabolism: Merck Manual Professional
		Genetic defects of the enzyme cause glucocerebroside accumulation in tissue macrophages through phagocytosis, forming Gaucher's cells.
		Patients receiving enzyme replacement require routine Hb and platelet monitoring; routine assessment of spleen and liver volume by CT or MRI; and routine assessment of bone disease by skeletal survey, dual-energy x-ray absorptiometry scanning, or MRI.
		Miglustat (100 mg po tid), a glucosylceramide synthase inhibitor, reduces glucocerebroside concentration (the substrate for glucocerebrosidase) and is an alternative for patients unable to receive enzyme replacement.
	www.merck.com /mmpe/print/sec19/ch296/ch296f.html (1858 words)

	Gaucher Disease - About disease (Site not responding. Last check: 2007-10-16)
		The enzyme glucocerebrosidase (pronounced "gloo-ko-ser-e-bro-si-dase) is located within the lysosomes and is responsible for breaking down glucocerebroside into glucose and a fat called ceramide.
		People with Gaucher disease lack the normal form of the glucocerebrosidase enzyme and are unable to break down glucocerebriside.
		This is why Gaucher disease is often referred to as a "storage disease".
	www.childrensgaucher.org /about/aboutdisease.htm (596 words)

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