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β-Glucocerebrosidase (also called acid β-glucosidase, D-glucosyl-N-acylsphingosine glucohydrolase, or GCase) is an enzyme with glucosylceramidase activity (EC 3.2.1.45) that cleaves by hydrolysis the β-glycosidic linkage of the chemical glucocerebroside, an intermediate in glycolipid metabolism that is abundant in cell membranes (particularly skin cells). [5]
Type III: muscle twitches known as myoclonus, convulsions, dementia, and ocular muscle apraxia; Parkinson's disease is recognized as being more common in Gaucher's disease patients and their heterozygous carrier relatives. [5] Osteoporosis: 75% of patients develop visible bony abnormalities due to the accumulated glucosylceramide.
It is a recombinant DNA-produced analogue of the human enzyme β-glucocerebrosidase. Cerezyme is a freeze-dried medicine containing imiglucerase, manufactured by Genzyme Corporation. It is given intravenously after reconstitution as a treatment for Type 1 and Type 3 [4] Gaucher's disease. It is available in formulations containing 200 or 400 ...
The term "steroid dementia" was coined by Varney et al. (1984) in reference to the effects of long-term glucocorticoid use in 1,500 patients. [3] While the condition generally falls under the classification of Cushing's syndrome , the term "steroid dementia syndrome" is particularly useful because it recognizes both the cause of the syndrome ...
In Gaucher's disease, the enzyme glucocerebrosidase is nonfunctional and cannot break down glucocerebroside into glucose and ceramide in the lysosome. [1] Affected macrophages, called Gaucher cells, have a distinct appearance similar to "wrinkled tissue paper" under light microscopy, because the substrates build-up within the lysosome.
Donepezil, sold under the brand name Aricept among others, is a medication used to treat dementia of the Alzheimer's type. [5] [6] [10] It appears to result in a small benefit in mental function and ability to function. [11]
Nicergoline, sold under the brand name Sermion among others, is an ergot derivative used to treat senile dementia and other disorders with vascular origins. Internationally it has been used for frontotemporal dementia as well as early onset in Lewy body dementia and Parkinson's dementia.
The systems of the body most affected by chemotherapy drugs include visual and semantic memory, attention and motor coordination and executive functioning. [9] [10] These effects can impair a chemotherapy patient's ability to understand and make decisions regarding treatment, perform in school or employment and can reduce quality of life. [10]