https://www.selleckchem.com/products/LY2784544.html A total of 23 genera from four phyla, namely Firmicutes, Proteobacteria, Bacteroidetes and Actinobacteria, were determined to be different between group T0 and group C, while only 8 genera were different between group T3 and group C. Repeated analysis of variance suggested a complex change during the low-fat diet treatment. The butyrate-producing bacteria Anaerotruncus exhibited a slight increase, while Roseburia was significantly increased at the T1 stage but then gradually decreased at the later stage. In summary, a low-fat diet was effective for patients with T2DM in reducing blood glucose and the BMI, and, to a certain extent, improving the intestinal flora to reach a normal composition. The study was registered in the Chinese Clinical Trial Registry (ChiCTR; registration no. ChiCTR1900028663).Mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase (HMGCS2) deficiency is a metabolic disorder caused by mutations in the HMGCS2 gene. The present study describes the identification of four cases of HMGCS2 deficiency in Japan. Hepatomegaly and severe metabolic acidosis were observed in all cases. Fatty liver was identified in three cases, which suggested the unavailability of fatty acids. All patients presented with a high C2/C0 ratio, suggesting that the fatty acid oxidation pathway was normal during metabolic crisis. Genetic analyses revealed five rare, novel variants (p.G219E, p.M235T, p.V253A, p.S392L and p.R500C) in HMGCS2. To confirm their pathogenicity, a eukaryotic expression system and a bacterial expression system was adopted that was successfully used to obtain affinity-purified HMGCS2 protein with measurable activity. Purified M235T, S392L and R500C proteins did not retain any residual activity, whilst the V253A variant showed some residual enzymatic activity. Judging from the transient expression experiment in 293T cells, the G219E variant appeared to be unstable. In conclusion, the present study identifie