Hepatocyte nuclear factor 4α (HNF4α) is a transcription factor that belongs to the steroid hormone receptor superfamily and is enriched in liver. HNF4α, in association with PGC-1α, activates gluconeogenic genes such as phosphoenolpyruvate carboxykinase and glucose-6-phosphatase genes in fasted livers. Conditional knockout of the HNF4α gene in the mouse liver destroys lipid homeostasis and leads to lipid accumulation in the liver and a reduction of serum cholesterol and triglyceride levels. Mutations in HNF4α have been linked to maturity-onset diabetes of the young (MODY).
For research use only. Not for human, diagnostic or therapeutic use.
Involvement In Disease
Maturity-onset diabetes of the young 1 (MODY1); Diabetes mellitus, non-insulin-dependent (NIDDM); Fanconi renotubular syndrome 4 with maturity-onset diabetes of the young (FRTS4)
Hepatocyte nuclear factor 4α (HNF4α) is a transcription factor that belongs to the steroid hormone receptor superfamily and is enriched in liver. HNF4α, in association with PGC-1α, activates gluconeogenic genes such as phosphoenolpyruvate carboxykinase and glucose-6-phosphatase genes in fasted livers. Conditional knockout of the HNF4α gene in the mouse liver destroys lipid homeostasis and leads to lipid accumulation in the liver and a reduction of serum cholesterol and triglyceride levels. Mutations in HNF4α have been linked to maturity-onset diabetes of the young (MODY).
This protein is a nuclear transcription factor which binds DNA as a homodimer. The encoded protein controls the expression of several genes, including hepatocyte nuclear factor 1 alpha, a transcription factor which regulates the expression of several hepatic genes. This gene may play a role in development of the liver, kidney, and intestines. Mutations in this gene have been associated with monogenic autosomal dominant non-insulin-dependent diabetes mellitus type I. Alternative splicing of this gene results in multiple transcript variants.
Transcriptionally controlled transcription factor. Binds to DNA sites required for the transcription of alpha 1-antitrypsin, apolipoprotein CIII, transthyretin genes and HNF1-alpha. May be essential for development of the liver, kidney and intestine.
Pathway
AMPK signaling
Protein Families
Nuclear hormone receptor family, NR2 subfamily
Buffer
Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Format
liquid
Purification
Affinity purification
Purity
Affinity purification
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Storage Buffer
Store at -20oC or -80oC. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.