Figure 8.

The effects of adipose FH deficiency. FH deficiency disrupts the Krebs cycle and leads to severe mitochondrial dysfunction with altered mitochondrial ultrastructure and low levels of ATP. Adipose FH deficiency in WAT of AFHKO mice causes a reduced capacity for TG synthesis, small cell diameter, low fat mass, and low basal plasma NEFA. In BAT, AFHKO mice show a marked decrease of UCP1 expression, hypertrophic brown adipocytes, and defective thermogenesis. At normal room temperature (21°C), these AFHKO mice are lean and protected against hepatic steatosis and insulin resistance. FAO, fatty acid oxidation; Fum, fumarate; Mal, malate; RC, respiratory chain.