Acid phosphatase in Gaucher's disease.
نویسندگان
چکیده
Increased acid phosphatase activity in the serum and tissues of patients with Gaucher's disease has now been recognized for two decades, but as yet no relation has been established between the enzyme and the etiology and progress of the disease. Here, we review results obtained by various investigators, ranging from a consideration of the methods used for the evaluation of serum acid phosphatase in Gaucher's disease to the most recent findings regarding the purification and characterization of two acid phosphatase isoenzymes from the spleen from patients with Gaucher's disease. We also discuss the intracellular location of tissue acid phosphatase in patients with Gaucher's disease and its contribution to the increased activity in serum.
منابع مشابه
Acid hydrolases in leukocytes and platelets of normal subjects and in patients with Gaucher's and Fabry's disease
Lymphocytes, monocytes, neutrophilic granulocytes and platelets were each separated to greater than 95% purity from six normal subjects, three patients with Gaucher's disease, two heterozygotes for Gaucher's disease, and one patient with Fabry's disease. Activities of the following acid hydrolases were determined: "acid" (pH 4.0) beta-glucosidase, pH 5.0 beta-glucosidase, alpha-galactosidase, a...
متن کاملGaucher's disease in the Cape coloured population of the RSA, including a family with 5 affected siblings.
Nine Cape Coloured children from 4 families with severe non-neuropathic Gaucher's disease are documented. The diagnosis was confirmed histologically in the bone marrow, spleen and liver, and by serum acid phosphatase and leucocyte beta-glucosidase assays. This represents a minimum prevalence for Gaucher's disease of 1 in 247,350 in this population and an approximate genetic carrier rate of 1 in...
متن کاملAntibodies to porcine uteroferrin used in measurement of human tartrate-resistant acid phosphatase.
The immunological similarity between human tartrate-resistant acid phosphatase (EC 3.1.3.2) and porcine uteroferrin previously reported for the isoenzyme from spleens of patients with leukemic reticuloendotheliosis (Ketcham et al., J Biol Chem 1985;260:5768-76) has been confirmed for partly purified acid phosphatase found in the spleen of a patient with Gaucher's disease, and for the correspond...
متن کاملClinical applicability of acid phosphatase isoenzyme assay.
We compared electrophoretic evaluation of acid phosphatase isoenzymes with spectrophotometric determination of prostatic acid phosphatase in terms of clinical utility. In all of 33 cases of prostatic carcinoma, an increased prostatic fraction was detected; in nine prostatectomized patients, this fraction returned to normal as measured by either technique. Abnormal spectrophotometric results wer...
متن کاملGaucher's disease of the lung causing severe pulmonary hypertension with associated acute recurrent pericarditis.
GAUCHER'S DISEASE is a familial disorder characterized by accumulation in reticuloendothelial cells of glucocerebrosides, compounds containing sphingosine, fatty acid, and glucose in equimolar amounts. The storage cells, called Gaucher cells, have a characteristic appearance, and their increasing numbers in the liver, spleen, lymph nodes, and bone marrow are responsible for most of the clinical...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- Clinical chemistry
دوره 26 3 شماره
صفحات -
تاریخ انتشار 1980