Phenotypic Characterization of Female Carrier Mice Heterozygous for Tafazzin Deletion.

Barth syndrome (BTHS) is caused by mutations in tafazzin resulting in deficits in cardiolipin remodeling that alter major metabolic processes. The tafazzin gene is encoded on the X chromosome, and therefore BTHS primarily affects males. Female carriers are typically considered asymptomatic, but age-related changes have been reported in female carriers of other X-linked disorders.

Phenotypic Characterization of Male -Knockout Mice at 3, 6, and 12 Months of Age.

Barth syndrome (BTHS) is an X-linked mitochondrial disease caused by mutations in the gene encoding for tafazzin (), a key enzyme in the remodeling of cardiolipin. Mice with a germline deficiency in have been generated (-KO) but not yet fully characterized. We performed physiological assessments of 3-, 6-, and 12-month-old male -KO mice, including measures of perinatal survival, growth, lifespan, gross anatomy, whole-body energy and substrate metabolism, glucose homeostasis, and exercise capacity.

Middle-Aged -Deficient Mice Have Altered Metabolic Measures.

Lysophosphatidic acid acyltransferases/acylglycerophosphate acyltransferases (LPAATs/AGPATs) are a group of homologous enzymes that catalyze the formation of phosphatidic acid (PA) from lysophosphatidic acid. We have previously reported that LPAATδ/AGPAT4 localizes to mitochondria, suggesting a potential role in energy metabolism. However, in prior studies of young -deficient mice (age 9-12 weeks old), we found no differences in body weights, food intakes, activity levels, respiratory gas exchange, or energy expenditure compared to their wildtype () littermates.

Effect of cannabidiol on apoptosis and cellular interferon and interferon-stimulated gene responses to the SARS-CoV-2 genes ORF8, ORF10 and M protein.

Aims: To study effects on cellular innate immune responses to ORF8, ORF10, and Membrane protein (M protein) from the Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) that causes COVID-19, in combination with cannabidiol (CBD).

Main Methods: HEK293 cells transfected with plasmids expressing control vector, ORF8, ORF10, or M protein were assayed for cell number and markers of apoptosis at 24 h, and interferon and interferon-stimulated gene expression at 14 h, with or without CBD. Cells transfected with polyinosinic:polycytidylic acid (Poly (I:C)) were also studied as a general model of RNA-type viral infection.

Glucagon-like Peptide-1 Secretion Is Inhibited by Lysophosphatidic Acid.

Glucagon-like peptide-1 (GLP-1) potentiates glucose-stimulated insulin secretion (GSIS). While dozens of compounds stimulate GLP-1 secretion, few inhibit. Reduced GLP-1 secretion and impaired GSIS occur in chronic inflammation.

Characterization of a novel transcript variant: implications for D6D activity regulation in cells.

Delta-6-desaturase (D6D) activity is deficient in MCF-7 and other cancer cell lines, but it is not explained by gene mutations. This deficient activity was not ameliorated by induction of the gene; therefore, we hypothesized that some of the induced transcript variants () may play a negative regulatory role. is the reference , coding for full-length D6D isoform 1 (D6D-iso1), and alternative transcriptional start sites result in and variants encoding D6D-iso2 and D6D-iso3 isoforms, respectively, which lack the catalytically critical N-terminal domain.