The role of preemptive liver transplantation in primary hyperoxaluria type 1

2005 ◽  
Vol 33 (5) ◽  
pp. 376-379 ◽  
Author(s):  
Markus J. Kemper
Keyword(s):  
Liver Transplantation ◽  
Primary Hyperoxaluria ◽  
Primary Hyperoxaluria Type 1 ◽  
Primary Hyperoxaluria Type ◽  
Hyperoxaluria Type

scholarly journals Liver Transplantation in Primary Hyperoxaluria Type 1: We Have to Find an Alternative!

2021 ◽  
Vol 105 (4) ◽  
pp. e46-e47
Author(s):  
Arnaud Devresse ◽  
Nathalie Godefroid ◽  
Blaise Anthonissen ◽  
Laura Labriola ◽  
Catherine de Magnée ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Primary Hyperoxaluria ◽  
Primary Hyperoxaluria Type 1 ◽  
Primary Hyperoxaluria Type ◽  
Hyperoxaluria Type

Long-term results of pre-emptive liver transplantation in primary hyperoxaluria type 1

2000 ◽  
Vol 4 (3) ◽  
pp. 177-181 ◽  
Author(s):  
Daniela Nolkemper ◽  
Markus J. Kemper ◽  
Martin Burdelski ◽  
Irith Vaismann ◽  
Xavier Rogiers ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Primary Hyperoxaluria ◽  
Long Term Results ◽  
Primary Hyperoxaluria Type 1 ◽  
Primary Hyperoxaluria Type ◽  
Hyperoxaluria Type

PRIMARY HYPEROXALURIA TYPE 1: IMPROVED OUTCOME WITH TIMELY LIVER TRANSPLANTATION: A SINGLE-CENTER REPORT OF 36 CHILDREN

2001 ◽  
Vol 72 (3) ◽  
pp. 428-432 ◽  
Author(s):  
Rivka Shapiro ◽  
Irit Weismann ◽  
Hana Mandel ◽  
Bela Eisenstein ◽  
Ziv Ben-Ari ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Primary Hyperoxaluria ◽  
Single Center ◽  
Primary Hyperoxaluria Type 1 ◽  
Primary Hyperoxaluria Type ◽  
Center Report ◽  
Improved Outcome ◽  
Hyperoxaluria Type

scholarly journals Inhibition of alanine:glyoxylate aminotransferase 1 dimerization is a prerequisite for its peroxisome-to-mitochondrion mistargeting in primary hyperoxaluria type 1.

1996 ◽  
Vol 135 (4) ◽  
pp. 939-951 ◽  
Author(s):  
J M Leiper ◽  
P B Oatey ◽  
C J Danpure
Keyword(s):  
Primary Hyperoxaluria ◽  
Mitochondrial Import ◽  
Primary Hyperoxaluria Type 1 ◽  
Whole Cells ◽  
Primary Hyperoxaluria Type ◽  
Alanine:Glyoxylate Aminotransferase ◽  
Hyperoxaluria Type

Peroxisome-to-mitochondrion mistargeting of the homodimeric enzyme alanine:glyoxylate aminotransferase 1 (AGT) in the autosomal recessive disease primary hyperoxaluria type 1 (PH1) is associated with the combined presence of a normally occurring Pro(11)Leu polymorphism and a PH1-specific Gly170Arg mutation. The former leads to the formation of a novel NH2-terminal mitochondrial targeting sequence (MTS), which although sufficient to direct the import of in vitro-translated AGT into isolated mitochondria, requires the additional presence of the Gly170Arg mutation to function efficiently in whole cells. The role of this mutation in the mistargeting phenomenon has remained elusive. It does not interfere with the peroxisomal targeting or import of AGT. In the present study, we have investigated the role of the Gly170Arg mutation in AGT mistargeting. In addition, our studies have led us to examine the relationship between the oligomeric status of AGT and the peroxisomal and mitochondrial import processes. The results obtained show that in vitro-translated AGT rapidly forms dimers that do not readily exchange subunits. Although the presence of the Pro(11)Leu or Gly170Arg substitutions alone had no effect on dimerization, their combined presence abolished homodimerization in vitro. However, AGT containing both substitutions was still able to form heterodimers in vitro with either normal AGT or AGT containing either substitution alone. Expression of various combinations of normal and mutant, as well as epitope-tagged and untagged forms of AGT in whole cells showed that normal AGT rapidly dimerizes in the cytosol and is imported into peroxisomes as a dimer. This dimerization prevents mitochondrial import, even when the AGT possesses an MTS generated by the Pro(11)Leu substitution. The additional presence of the Gly170Arg substitution impairs dimerization sufficiently to allow mitochondrial import. Pharmacological inhibition of mitochondrial import allows AGT containing both substitutions to be imported into peroxisomes efficiently, showing that AGT dimerization is not a prerequisite for peroxisomal import.

LIVER TRANSPLANTATION IN PRIMARY HYPEROXALURIA TYPE 1

The Lancet ◽  
1989 ◽  
Vol 333 (8647) ◽  
pp. 1142-1143 ◽  
Author(s):  
Pierre Cochat ◽  
Jean Louis Faure ◽  
Priscille Divry ◽  
ChristopherJ. Danpure ◽  
Bruno Descos ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Primary Hyperoxaluria ◽  
Primary Hyperoxaluria Type 1 ◽  
Primary Hyperoxaluria Type ◽  
Hyperoxaluria Type

Early Renal Failure After Domino Liver Transplantation Using Organs From Donors With Primary Hyperoxaluria Type 1

2010 ◽  
Vol 90 (7) ◽  
pp. 782-785 ◽  
Author(s):  
Fuat H. Saner ◽  
Juergen Treckmann ◽  
Johann Pratschke ◽  
Helmut Arbogast ◽  
Axel Rahmel ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Renal Failure ◽  
Primary Hyperoxaluria ◽  
Primary Hyperoxaluria Type 1 ◽  
Primary Hyperoxaluria Type ◽  
Domino Liver Transplantation ◽  
Hyperoxaluria Type
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