Enhancement of Glutathione Content in Glutathione Synthetase-Deficient Fibroblasts from a Patient with 5-Oxoprolinuria via Metabolic Cooperation with Normal Fibroblasts

1994 ◽  
Vol 212 (1) ◽  
pp. 69-76 ◽  
Author(s):  
Terrance J. Kavanagh ◽  
Ganesh Raghu ◽  
Collin C. White ◽  
George M. Martin ◽  
Peter S. Rabinovitch ◽  
...  
Author(s):  
J.S. Ryerse

Gap junctions are intercellular junctions found in both vertebrates and invertebrates through which ions and small molecules can pass. Their distribution in tissues could be of critical importance for ionic coupling or metabolic cooperation between cells or for regulating the intracellular movement of growth control and pattern formation factors. Studies of the distribution of gap junctions in mutants which develop abnormally may shed light upon their role in normal development. I report here the distribution of gap junctions in the wing pouch of 3 Drosophila wing disc mutants, vg (vestigial) a cell death mutant, 1(2)gd (lethal giant disc) a pattern abnormality mutant and 1(2)gl (lethal giant larva) a neoplastic mutant and compare these with wildtype wing discs.The wing pouch (the anlagen of the adult wing blade) of a wild-type wing disc is shown in Fig. 1 and consists of columnar cells (Fig. 5) joined by gap junctions (Fig. 6). 14000x EMs of conventionally processed, UA en bloc stained, longitudinally sectioned wing pouches were enlarged to 45000x with a projector and tracings were made on which the lateral plasma membrane (LPM) and gap junctions were marked.


Blood ◽  
1983 ◽  
Vol 62 (4) ◽  
pp. 754-757 ◽  
Author(s):  
JT Prchal ◽  
WM Crist ◽  
M Roper ◽  
VP Wellner

Abstract The clinical and laboratory features of a 3-mo-old black male infant with glutathione (GSH) synthetase deficiency of the generalized type was evaluated. Partial albinism, brisk hemolytic anemia, recurrent febrile episodes, and mental retardation were noted. Also, severe recurrent metabolic acidosis and marked oxoprolinemia and oxoprolinuria were found in the proband but not in his first-degree relatives. The relationship of these disease manifestations to the underlying metabolic defect is discussed.


2021 ◽  
Vol 33 (8) ◽  
pp. 1546-1564
Author(s):  
Gilles Bonvento ◽  
Juan P. Bolaños

Author(s):  
Sonja Blasche ◽  
Yongkyu Kim ◽  
Ruben A. T. Mars ◽  
Daniel Machado ◽  
Maria Maansson ◽  
...  

2005 ◽  
Vol 2 (3) ◽  
pp. 375-381 ◽  
Author(s):  
I. R. Punitha ◽  
K. Rajendran ◽  
Arun Shirwaikar ◽  
Annie Shirwaikar

Alcoholic extract of the stems ofCoscinium fenestratum, a medicinal plant indigenous to India and Sri Lanka used in ayurveda and siddha medicine for treating diabetes, was studied for its carbohydrate metabolism effect and antioxidant status in streptozotocin–nicotinamide induced type 2 diabetic rats. Oral administration ofC. fenestratumstem extract in graded doses caused a significant increase in enzymatic antioxidants such as catalase, superoxide dismutase, glutathione synthetase, peroxidase, and glutathione peroxidase and in the nonenzymatic antioxidants ascorbic acid, ceruloplasmin and tocopherol. Effects of alcoholic extract on glycolytic enzymes such as glucose-6-phosphate dehydrogenase, lactate dehydrogenase and hexokinase showed a significant increase in their levels, whereas a significant decrease was observed in the levels of gluconeogenic enzyme, glucose-6-phosphatase and alanine aminotransferase in treated diabetic rats. Serum creatinine and urea levels also declined significantly. This investigation demonstrates significant antidiabetic activity ofC. fenestratum.


Science ◽  
1979 ◽  
Vol 206 (4422) ◽  
pp. 1089-1091 ◽  
Author(s):  
L. Yotti ◽  
C. Chang ◽  
J. Trosko

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