Gaucher Acid phosphatase

What is the cause of elevation in serum acid phosphatase (ACP) activity in Gaucher disease ... 

The slight but definite elevations of serum acid phosphatase activity in conditions such as thrombocytopenia (02, Zl), Gaucher s disease (T6, T8), or various myeloproliferative diseases (B6) indicate the possibility that platelets, the marrow, and the reticuloendothelial system may also be sources of acid phosphatase. These aspects will be discussed more fully later in the review. 

In 1957, Tuchman and Swick (T6) reported the finding of elevated serum acid phosphatase activities, ranging from 7.0 to 10.3 K.A. units in a 68-year-old man, who was first suspected of having carcinoma of the prostate. The diagnosis of Gaucher s disease was then considered and confirmed by a sternal marrow aspiration. By 1959, Tuchman et al. (T7)... 

Isoenzymes of Acid Phosphatase in Serum and Spleen Cell Suspensions of Gaucher s Disease... 

Although serum acid phosphatase activity has already been considered in certain diseases of childhood, such as leukemia or Gaucher s disease, it may be of value to make several general comments in this area. Laron and Kowadlo (L4) found that the mean normal values for total serum acid phosphatase were 5.23 1.26 K.A. units for children 1 year of age and 4.63 0.93 K.A. units for children 2-8 years of age. These are higher than the values for adults. The mean value for l- (-I-) -tartrate-inhibited acid phosphatase activity was 0.21 0.28 K.A. units for all age groups. 

G6. Goldberg, A. F., Takakura, K., and Rosenthal, R. L., Electrophoretic separation of serum acid phosphatase isoenzymes in Gaucher s disease, prostatic carcinoma and multiple myeloma. Nature London) 211, 41-43 (1966). 

T6. Tuehman, L. R., and Swick, M., High acid phosphatase level indicating Gaucher s disease in patient with prostatism. J. Amer. Med. Ass. 164, 2034-2035 (1957). 

T9. Tyson, M. C., Grossman, W. I., and Tuehman, L. R., Gaucher s disease (with elevated serum acid phosphatase level) masquerading as cirrhosis of the liver. Amer. J. Med. 37, 156-158 (1964). 

A comparison with the acid phosphatase (s) which may be increased in carcinoma of the prostate, Paget s disease, certain liver diseases, and hyperparathyroidism showed that the acid phosphatase of Gaucher s disease can be differentiated from the former with the use of various activators and inhibitors (Grundig et al. 1965). In contrast to prostatic phosphatases it is not inhibited by L-tartrate... 

Abul-Fadl and King 1949, Tuchman et al. 1959). While normally a fraction of serum phosphatase activity can result from thrombocytes and red cells, this source has been excluded for the hyperphosphatasia of GD by demonstration of different reactions to inhibitors (Hillborg and Estborn 1964). The same authors even found decreased activity of the acid phosphatase in red cells of Gaucher patients. 

A search for elevated serum phosphatase in healthy relatives of Gaucher patients has been unsuccessful neither parents nor siblings of affected subjects had abnormal values (Hillborg and Estborn 1964). Measurement of the acid phosphatase is therefore not a potential test for heterozygous subjects. 

Hillborg, P.-O., and B. Estborn Acid phosphatase activity of serum, thrombocytes and erythrocytes in a juvenile form of Gaucher s disease. Acta paediat. (Uppsala) 53,558 (1964). 

Tuchman, L. R., G. Goldstein, and M. Clyman Studies on the nature of the increased serum acid phosphatase in Gaucher s disease. Amer. J. Med. 27, 959 (1959). 

H. SuNA, and J. J. Carr Elevation of serum acid phosphatase in Gaucher s Disease. J. Mt. Sinai Hosp. 23, 227 (1956). 

There are a number of findings, the relation of which to the disease has not been established. A ten-fold elevation of serum acid phosphatase has been observed in a three year-old patient by Hastrup and Videbaek (1954). Since this enzyme differed from prostatic phosphatase by exhibiting a lower activity towards beta-glycerophosphate, it was similar to the acid phosphatase which is found elevated in Gaucher s disease and there is reason to doubt the diagnosis of NPD in this case. Elevation of serum acid phosphatase activity in NPD has not been found by other observers for instance the relevant values were normal on 11 occasions in six patients studied by Crocker and Farber (1958). 

In addition to its archetypical members, uteroferrin and bovine spleen add phosphatase, the class of purple add phosphatases includes proteins isolated from rat bone and. spleen spleens of patients with Gaucher s disease or leukemic reticuloen-dotheliosis equine uterine flushings bovine cortical bone giant ceU tumors human placenta and microorganisms . The plant enzymes include an Fe-Zn phosphatase from red kidney beans and an Fe-Fe or Mn(in) protein from sweet potato tubers . Although less well-defined and more heterogeneous than their mammalian counterparts, the color and iron content of the plant enzymes warrant their designation as purple acid phosphatases. 

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