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. 2019 Jun 26;10:411. doi: 10.3389/fendo.2019.00411

Table 1.

Summary of loss-of-function mouse models revealing liver-specific corepressor functions and target TFs.

Protein name (gene name) Mouse model Key features of the phenotype Target TF References
NCOR (Ncor1) Global KO • Embryonic lethality at E15.5
• Impaired erythroid, thymocyte, and CNS development
(17)
Liver NCORi transgenic • Overexpression of a dominant-negative NCOR blocked basal transcription of TR-responsive genes but had no effect on ligand-activation
• Increased endogenous SMRT and NCOR mRNA expression
• Increased hepatocyte proliferation in euthyroid mice
TR (18)
Liver NCOR
ΔRID knockin
• Increased expression of TR positive targets in both hypothyroid and euthyroid conditions
• Improved cholesterol tolerance due to diminished intestinal cholesterol absorption (as the result of changes in the composition and hydrophobicity of the bile salt pool)
TR (1921)
Global NCOR
ΔRID knockin
• Increased energy expenditure as a result of enhanced sensitivity to TH
• Could rescue insulin-resistant phenotype of mutant TRβ
TR (22, 23)
Global DADm knockin • Leaner due to increased energy expenditure
• Improved insulin-sensitivity
• Abnormal circadian behavior due to aberrant regulation of clock genes
• Altered oscillatory patterns of several metabolic genes
• Derepressed TH-activated genes in euthyroid and hypothyroid mice liver
TR
LXR
(24, 25)
LKO
(AAV8-TBG-Alb-Cre)
• Developed hepatosteatosis due to increased lipogenesis RevErb
LXR
(26)
LKO
(Alb-Cre)
• Repressed lipid synthesis in the fasting state
• Repressed fatty acid oxidation and ketogenesis in the feeding state
• Improved liver regeneration after partial hepatectomy and blocked diethylnitrosamine (DEN)-induced hepatocarcinogenesis
PPARα
LXR
ERRα
(27, 28)
LKO
(Alb-Cre)
• Developed hepatosteatosis due to increased lipogenesis TR (29)
SMRT
(Ncor2)
Global KO • Embryonic lethality before E16.5 due to lethal heart defect
• Impaired neural development in forebrain
• Fail to maintenance of the neural stem cell state
RAR (30)
Global SMRT mRID knockin • Decreased energy expenditure
• Developed global glucose tolerance and insulin sensitivity
• Increased adiposity due to enhanced adipogenesis
• Impaired type I pneumocytes differentiation and produced respiratoty distress syndrome at birth
TR
PPARγ
(31)
Global SMRTmRID1 knockin • Accelerated aging (reduced mitochondrial function and increased susceptibility to oxidative stress)
• Developed global glucose intolerance and insulin resistance
• Upon HFD:
• Obesity, insulin-insensitive, and refractory to the glucose lowering effects of TZD and AICAR, energy metabolism shifts from OxPhos to glycolysis
• Mesenteric adipose tissue: adipocyte hypertrophy and increased inflammatory
• Liver: hepatosteatosis
• BAT: reduced thermogenic capacity and mitochondrial biogenesis
RAR
TR PPARα
PPARγ
(32, 33)
LKO
(AAV8-TBG-Alb-Cre)
• No obvious metabolic phenotype (26)
LKO
(Alb-Cre)
• Little effect on most of TR targets in either euthyroid or hypothyroid animals
• de-repressed RAR targets (Cyp26a1)
RAR (29)
NCOR/SMRT
(Ncor1/2)
NCOR/SMRT LKO (Alb-Cre) • Hepatosteatosis due to activated hepatic lipogenesis and lipid storage
• Normal glucose sensitivity
• Increased ChREBP isoforms expression
TR
RAR
(29)
Global NS-DADm knockin • Upregulated lipid-metabolic genes and mile hepatosteatosis
• Undetectable HDAC3 enzyme activity, abrogated genome-wide HDAC3 recruitment, as well as increased local histone acetylation level
Lipid-sensing
NRs
(34)
HDAC3
(Hdac3)
Global KO • Embryonic lethality before E9.5 (35)
LKO
(Mx1-Cre plus pIpC injection or Alb-Cre)
• Hepatomegaly due to hepatocyte hypertrophy
• Hepatosteatosis
• Increased serum TG, total serum cholesterol, and LDL
• Hypersensitive to insulin
PPARγ2 (36)
LKO
(AAV8-TBG-Alb-Cre)
• Alteration in circadian genes
• Hepatosteatosis due to increased lipogenesis and sequestration
• Repressed gluconeogenesis
• Improved insulin sensitivity
RevErb
HNF4α
HNF6
(26, 34, 3741)
TBL1
(Tbl1x)TBLR1 (Tbl1xr1)
Liver RNAi knockdown (adenovirus- delivered shRNA) • Hepatosteatosis
• Highly elevated VLDL TG
• Inhibited of PPARα activity under both normal and HFD conditions
PPARα (42)
GPS2
(Gps2)
Global KO • Embryonic lethality around E10 (43)
LKO
(Alb-Cre)
• Dramatically reduced VLDL TG
• Protected from HFD-induced hepatic steatosis and MCD-induced fibrosis
• Enhanced PPARα-induced fatty acid oxidation
PPARα (44)
PROX1
(Prox1)
Liver RNAi knockdown (AAV8-TBG-shRNA) • Significantly elevated hepatic TG HNF4 (41)
LKO
(Alb-Cre)
• Hepatic injury
• Non-obese but insulin-resistant
• Suppressed glycolysis
• Upregulated both oxidative phosphorylation and autophagy
Lipid-sensing
NRs
(45)
RIP140
(Nrip1)
Global KO • Lean and resistance to HFD-induced obesity and hepatic steatosis
• Increased oxygen consumption
• Unaffected adipogenesis, increased certain lipogenic enzymes, and UCP1 in Adipose tissue
• Inhibited lipogenesis and enhanced gluconeogenesis
LXR (46, 47)
Liver RNAi knockdown
(adenovirus- delivered shRNA)
• Alleviated hepatic steatosis in tumor-bearing, cachectic animals
• Increased free fatty acid oxidation and ketogenesis
• Enhanced VLDL secretion
• Reduced peripheral lipoprotein lipase activity
LXR
PPARα
(48)
SHP
(Nr0b2)
Global KO • Increased bile acids synthesis under chow diet
• Dietary bile acids induce liver damage and restore feedback regulation
• A synthetic FXR agonist is not hepatotoxic and has no regulatory effects
• Cholestyramine enhanced the expression of CYP7A1 and CYP8B1 and reduced the bile acid pool
LRH1
HNF4α
LXR
(49)
Global KO • Increased bile acids synthesis under chow diet
• No significant defects in cholesterol metabolism under chow diet
• Bile acids still can suppress Cyp7a1 expression
• Resistant to bile acid induced liver damage
LRH1
HNF4α
LXR
(50, 51)
Liver-specific overexpression • Depleted of hepatic bile acid pool
• Accumulated TG in liver
FXR LRH1
HNF4α
PPARγ
SREBP1c
(52)
LKO
(Alb-Cre)
• De-repressed Cyp7a1 and Cyp8b1 under chow diet and cholesterol and cholic acid diet
• Resistance to diet induced hypercholesterolemia
Multiple NRs (53)
FXR SHP DLKO • Cholestasis and liver injury as early as 3 weeks of age due to dysregulation of bile acid homeostatic genes
• Activated C21 steroid biosynthesis pathway
• Lower hepatic TG accumulation, improved glucose/insulin tolerance, and accelerated fatty acid use in aged mice
Multiple NRs (54, 55)