Related Experiment Video
Updated: Jul 22, 2026

Generation of a Rat Model of Acute Liver Failure by Combining 70% Partial Hepatectomy and Acetaminophen
Published on: November 27, 2019
Hepatocyte nuclear factor 1alpha controls the expression of terminal complement genes.
M Pontoglio1, M Pausa, A Doyen
1Unité des Virus Oncogènes, Centre National de la Recherche Scientifique URA 1644, Département de Biotechnologie, Institut Pasteur, 75724 Paris cedex 15, France. marcop@pasteur.fr
Hepatocyte nuclear factor 1-alpha (HNF1alpha) is crucial for producing complement proteins C5 and C8A, essential for the membrane attack complex (MAC) in host defense. Its absence severely impairs complement activity and host defense mechanisms.
Area of Science:
- Immunology
- Molecular Biology
- Genetics
Background:
- The terminal complement system forms the membrane attack complex (MAC) for host defense.
- Liver synthesis of complement proteins is key, but tissue-specific expression control is unclear.
Purpose of the Study:
- Investigate the role of hepatocyte nuclear factor 1-alpha (HNF1alpha) in regulating terminal complement component gene expression in the liver.
- Determine the impact of HNF1alpha deficiency on MAC formation and complement-mediated hemolytic activity.
Main Methods:
- Analysis of C5 and C8A gene transcription in HNF1alpha-deficient mice.
- Quantitative assessment of other terminal complement component mRNAs (C8beta, C8gamma, C9).
- Complement activity assessment using a reconstitution assay with human sera and HNF1alpha-deficient mouse sera.
Main Results:
- HNF1alpha deficiency abrogated C5 and C8A gene transcription.
- Reduced mRNA levels for C8beta, C8gamma, and C9 were observed.
- HNF1alpha-deficient mouse sera exhibited negligible hemolytic activity, particularly affecting C5 and C8alpha-gamma subunits.
- C8beta activity was severely impacted, mirroring findings in human C8alpha-gamma deficiency.
Conclusions:
- HNF1alpha is essential for the hepatic expression of C5 and C8A genes.
- HNF1alpha plays a critical role in the assembly of the membrane attack complex (MAC).
- Disruption of HNF1alpha function significantly compromises complement-mediated host defense.
More Related Videos
07:09Hepatic Progenitor Specification from Pluripotent Stem Cells using a Defined Differentiation System
Published on: May 10, 2020
11:34A Competent Hepatocyte Model Examining Hepatitis B Virus Entry through Sodium Taurocholate Cotransporting Polypeptide as a Therapeutic Target
Published on: May 10, 2022
Related Concept Videos
Transcription Factors
Cell Specific Gene Expression
Regulation of Nuclear Protein Sorting
NF-κB-dependent Signaling Pathway
NF-κB-dependent Signaling Mechanism
The heterodimer of NF-κB...
General Transcription Factors
Cirrhosis II: Pathophysiology