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Volume 130, Issue 4, Pages 1317-1323 (April 2006)
Sustained Engraftment and Tissue Enzyme Activity After Liver Cell Transplantation for Argininosuccinate Lyase Deficiency Xavier Stéphenne, Mustapha Najimi, Catherine Sibille, Marie–Cécile Nassogne, Françoise Smets, Etienne M. Sokal Gastroenterology Volume 130, Issue 4, Pages (April 2006) DOI: /j.gastro Copyright © 2006 American Gastroenterological Association Institute Terms and Conditions
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Figure 1 This 42-month-old ASL-deficient patient received 11 cell infusions over a period of 5 months (black arrows: cryopreserved cells, red arrows: fresh isolated cells). The ammonia levels summarized 3.5 years before LCT decreased significantly after the infusions. A period of fluctuating ammonia levels was observed during 4 months, but then ammonia levels normalized during the last 6 months of follow-up. Black lines symbolize the different biopsies. Gastroenterology , DOI: ( /j.gastro ) Copyright © 2006 American Gastroenterological Association Institute Terms and Conditions
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Figure 2 (A and B) FISH Y chromosome on liver cells obtained by biopsy performed 2.5 months post-LCT in the ASL-deficient patient showing 4.7% of XXYY cells. Red: SRY (chromosome Y) and green: DXZ1 (chromosome X-CEP X). (C) Hybridization with centromeric probes specific of autosomes (CEP12, CEP16) of the 2.5-month biopsy fingerprints where XXYY cells were found in significant proportion, confirming the tetraploid status of XXYY cells. Red: CEP12 (chromosome 12) and green: CEP16 (chromosome 16). Gastroenterology , DOI: ( /j.gastro ) Copyright © 2006 American Gastroenterological Association Institute Terms and Conditions
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Figure 3 (A and B) FISH Y-18 chromosomes on liver cells obtained by biopsy performed 8 months post-LCT in the ASL-deficient patient indicating a significant percentage of 2N XXY, 2N XXYY. Red: SRY (chromosome Y), green: DXZ1 (chromosome X-CEP X), and blue: CEP 18 (chromosome 18). Gastroenterology , DOI: ( /j.gastro ) Copyright © 2006 American Gastroenterological Association Institute Terms and Conditions
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Figure 4 (A) Albumin (in green) and CK-7 (in red) staining of cell fingerprints from Y-positive biopsies indicating a 95% panel of albumin-positive, CK-7 negative cells (4′,6-diamidino-2-phenylindole nuclei staining in blue). (B) Five percent of cells being albumin negative and CK-7 positive on cell fingerprints from Y-positive biopsies (4′,6-diamidino-2-phenylindole nuclei staining in blue). Gastroenterology , DOI: ( /j.gastro ) Copyright © 2006 American Gastroenterological Association Institute Terms and Conditions
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