Hepatocyte Transplantation Ameliorates the Metabolic Abnormality in a Mouse Model of Acute Intermittent Porphyria
Acute intermittent porphyria (AIP) is an autosomal dominant disorder characterized by insufficient porphobilinogen deaminase (PBGD) activity. When hepatic heme synthesis is induced, porphobilinogen (PBG) and 5-aminolevulinic acid (ALA) accumulate, which causes clinical symptoms such as abdominal pai...
Main Authors: | , , , , , |
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Format: | Article |
Language: | English |
Published: |
SAGE Publishing
2014-09-01
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Series: | Cell Transplantation |
Online Access: | https://doi.org/10.3727/096368913X666980 |