Ornithine transcarbamylase and arginase I deficiency are responsible for diminished urea cycle function in the human hepatoblastoma cell line HepG2

2007 ◽  
Vol 39 (3) ◽  
pp. 555-564 ◽  
Author(s):  
Demetra Mavri-Damelin ◽  
Simon Eaton ◽  
Leonard H. Damelin ◽  
Myrddin Rees ◽  
Humphrey J.F. Hodgson ◽  
...  
Keyword(s):  
Cell Line ◽  
Urea Cycle ◽  
Ornithine Transcarbamylase ◽  
Arginase I ◽  
Hepatoblastoma Cell Line

scholarly journals Regulation of erythropoietin production in a human hepatoblastoma cell line

Blood ◽  
1987 ◽  
Vol 70 (6) ◽  
pp. 1904-1909
Author(s):  
OJ Nielsen ◽  
SJ Schuster ◽  
R Kaufman ◽  
AJ Erslev ◽  
J Caro
Keyword(s):  
Cell Line ◽  
Northern Blot ◽  
Proliferative Activity ◽  
Northern Blot Analysis ◽  
Cobalt Chloride ◽  
Biologically Active ◽  
Erythropoietin Production ◽  
Rna Probe ◽  
Hepatoblastoma Cell Line

Production of immuno and biologically active erythropoietin was documented to occur in the human hepatoblastoma cell line HepG-2. The expression of the erythropoietin gene was further verified by Northern blot analysis using a single stranded RNA probe. In vitro studies showed that erythropoietin production by these cells was not stimulated by hypoxia or cobalt chloride, but was related to the proliferative activity of the cells in culture. In addition it was found that the secretion of erythropoietin was almost completely abrogated by tunicamycin, an inhibitor of N-linked glycosylation. This effect of tunicamycin was also observed in a permanently transfected cell line that secretes erythropoietin in large quantities.

scholarly journals Generation of a Mouse Model for Arginase II Deficiency by Targeted Disruption of the Arginase II Gene

2001 ◽  
Vol 21 (3) ◽  
pp. 811-813 ◽  
Author(s):  
Ou Shi ◽  
Sidney M. Morris ◽  
Huda Zoghbi ◽  
Carl W. Porter ◽  
William E. O'Brien
Keyword(s):  
Urea Cycle ◽  
Elevated Plasma ◽  
Wild Type ◽  
Targeted Disruption ◽  
Arginase Ii ◽  
Plasma Arginine ◽  
Physiologic Role ◽  
Deficient Mice ◽  
Arginase I

ABSTRACT Mammals express two isoforms of arginase, designated types I and II. Arginase I is a component of the urea cycle, and inherited defects in arginase I have deleterious consequences in humans. In contrast, the physiologic role of arginase II has not been defined, and no deficiencies in arginase II have been identified in humans. Mice with a disruption in the arginase II gene were created to investigate the role of this enzyme. Homozygous arginase II-deficient mice were viable and apparently indistinguishable from wild-type mice, except for an elevated plasma arginine level which indicates that arginase II plays an important role in arginine homeostasis.

Characteristics of NO cycle coupling with urea cycle in non-hyperammonemic carriers of ornithine transcarbamylase deficiency

2013 ◽  
Vol 109 (3) ◽  
pp. 251-254 ◽  
Author(s):  
Hironori Nagasaka ◽  
Tohru Yorifuji ◽  
Hiroto Egawa ◽  
Ayano Inui ◽  
Tomoo Fujisawa ◽  
...  
Keyword(s):  
Urea Cycle ◽  
Ornithine Transcarbamylase ◽  
Ornithine Transcarbamylase Deficiency

Reduced T3 deiodination by the human hepatoblastoma cell line HepG2 caused by deficient T3 sulfation

1993 ◽  
Vol 1157 (1) ◽  
pp. 114-118 ◽  
Author(s):  
Paul G.J. van Stralen ◽  
Hans J. van der Hoek ◽  
Roel Docter ◽  
Marion de Jong ◽  
Eric P. Krenning ◽  
...  
Keyword(s):  
Cell Line ◽  
Hepatoblastoma Cell Line
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