Publications by authors named "J Krijt"
Article Synopsis
- Cystathionine β-synthase (CBS)-deficient homocystinuria (HCU) is a genetic disorder affecting sulfur amino acid metabolism, leading to various health complications and underscoring the need for better understanding of its biological processes.
- In a study involving a transgenic mouse model (I278T), researchers found significant metabolic imbalances, altered liver proteome, and changes in sphingolipid metabolism, although mitochondrial function appeared normal.
- A methionine-restricted diet (MRD) was shown to improve metabolic balance and reduce liver proteome disruptions in I278T mice, suggesting potential therapeutic benefits for HCU.
Background: In animals, dietary sulfur amino acid restriction (SAAR) improves metabolic health, possibly mediated by altering sulfur amino acid metabolism and enhanced anti-obesogenic processes in adipose tissue.
Aim: To assess the effects of SAAR over time on the plasma and urine SAA-related metabolites (sulfurome) in humans with overweight and obesity, and explore whether such changes were associated with body weight, body fat and adipose tissue gene expression.
Methods: Fifty-nine subjects were randomly allocated to SAAR (∼2 g SAA, n = 31) or a control diet (∼5.
Article Synopsis
- Regulation of hydrogen sulfide (HS) homeostasis in humans is not well understood, prompting a study of patients with rare enzyme deficiencies related to HS synthesis and catabolism.
- Analysis of sulfur compounds in these patients revealed unexpected results, such as increased bioavailable sulfide levels in those with cystathionine β-synthase (CBS) deficiency, suggesting compensatory mechanisms at play.
- The study highlights the complexity of HS regulation, showing that various genetic defects can lead to altered levels of sulfur compounds, underscoring the need for a thorough understanding of HS homeostasis in metabolic disorders.
The purpose of the study was to investigate the expression of ferroportin protein following treatments that affect systemic hepcidin. Administration of erythropoietin to C57BL/6J mice decreased systemic hepcidin expression; it also increased heart ferroportin protein content, determined by immunoblot in the membrane fraction, to approximately 200% of control values. This increase in heart ferroportin protein is very probably caused by a decrease in systemic hepcidin expression, in accordance with the classical regulation of ferroportin by hepcidin.
View Article and Find Full Text PDFThe chicken Tva cell surface protein, a member of the low-density lipoprotein receptor family, has been identified as an entry receptor for avian leukosis virus of classic subgroup A and newly emerging subgroup K. Because both viruses represent an important concern for the poultry industry, we introduced a frame-shifting deletion into the chicken locus with the aim of knocking-out Tva expression and creating a virus-resistant chicken line. The knock-out was prepared by CRISPR/Cas9 gene editing in chicken primordial germ cells and orthotopic transplantation of edited cells into the testes of sterilized recipient roosters.
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