Title: Adult human liver mesenchymal progenitor cells express phenylalanine hydroxylase
Authors: Baruteau, Julien
Nyabi, Omar
Najimi, Mustapha
Fauvart, Maarten
Sokal, Etienne # ×
Issue Date: 8-Apr-2014
Publisher: Freund Pub. House
Series Title: The Journal of Pediatric Endocrinology vol:27 issue:9-10 pages:863-868
Abstract: Phenylketonuria (PKU) is one of the most prevalent inherited metabolic diseases and is accountable for a severe encephalopathy by progressive intoxication of the brain by phenylalanine. This results from an ineffective L-phenylalanine hydroxylase enzyme (PAH) due to a mutated phenylalanine hydroxylase gene (PAH). Neonatal screening programs allow an early dietetic treatment with restrictive phenylalanine intake. This diet prevents most of the neuropsychological disabilities but remains challenging for lifelong compliance. Adult-derived human liver progenitor cells (ADHLPC) are a pool of precursors that can differentiate into hepatocytes. We aim to study PAH expression and PAH activity in differenciated ADHLPC. ADHLPC were isolated from human hepatocyte primary culture of 2 different donors and differenciated under specific culture conditions. We demonstrated the high expression of PAH and a large increase of PAH activity in differenciated LPC. The age of the donor, the cellular viability after liver digestion and cryopreservation affects PAH activity. ADHLPC might therefore be considered as a suitable source for cell therapy in PKU.
ISSN: 0334-018X
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Centre of Microbial and Plant Genetics
× corresponding author
# (joint) last author

Files in This Item:

There are no files associated with this item.

Request a copy


All items in Lirias are protected by copyright, with all rights reserved.

© Web of science