Isolation and characterization of a tartrate-sensitive splenic acid phosphatase in Gaucher's disease

1980 ◽  
Vol 205 (1) ◽  
pp. 156-168 ◽  
Author(s):  
Douglas B. Robinson ◽  
Robert H. Glew
1980 ◽  
Vol 26 (3) ◽  
pp. 371-382
Author(s):  
D B Robinson ◽  
R H Glew

Abstract 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.


1980 ◽  
Vol 26 (3) ◽  
pp. 371-382 ◽  
Author(s):  
D B Robinson ◽  
R H Glew

Abstract 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.


1987 ◽  
Vol 33 (10) ◽  
pp. 1832-1836 ◽  
Author(s):  
Z O Echetebu ◽  
T M Cox ◽  
D W Moss

Abstract 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 corresponding isoenzyme in other tissues and serum. Anti-uteroferrin antibodies raised in rabbits have been used to demonstrate the feasibility of their application in an immunoassay for tartrate-resistant acid phosphatase in serum.


The Lancet ◽  
1968 ◽  
Vol 292 (7563) ◽  
pp. 358-359 ◽  
Author(s):  
G.P. Perona ◽  
C. Baccichetti ◽  
R. Tenconi

Genetics ◽  
1992 ◽  
Vol 130 (3) ◽  
pp. 445-449
Author(s):  
A M Schweingruber ◽  
H Fankhauser ◽  
J Dlugonski ◽  
C Steinmann-Loss ◽  
M E Schweingruber

Abstract Mutants from Schizosaccharomyces pombe deficient in the regulation of thiamine-repressible acid phosphatase have been isolated. Mutants expressing derepressed levels of the enzyme in the presence and absence of thiamine map in three genes, tnr1, tnr2 and tnr3. mRNA levels of the pho4 gene (coding for thiamine repressible acid phosphatase) and another thiamine-regulatable gene, thi3 (coding for a thiamine biosynthetic enzyme and corresponding to nmt1) are constitutively synthesized in the mutants. The mutants also exhibit constitutive thiamine transport which is thiamine repressible in wild type. The tnr3 mutants reveal a 10-20-fold higher intracellular thiamine level than tnr1 and tnr2 mutants and wild type. Mutants expressing repressed levels of thiamine-repressible acid phosphatase map in gene thi1. No or little amounts of pho4- and nmt1-specific mRNA can be detected. These mutants are impaired in thiamine uptake and are thiamine auxotrophic due to the inability to synthesize the thiazole moiety of the thiamine molecule. All tested tnr and thi1 alleles are recessive, and thi1 mutations are epistatic over tnr mutations. We assume that the thi1 and tnr genes are involved in thiamine-mediated transcription control.


1973 ◽  
Vol 113 (2) ◽  
pp. 727-738 ◽  
Author(s):  
Akio Toh-e ◽  
Yoshinami Ueda ◽  
Sei-Ichiro Kakimoto ◽  
Yasuji Oshima

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