Acylcarnitines are fatty acid metabolites that play important roles in many cellular energy
metabolism pathways. They have historically been used as important diagnostic markers for …

Leigh syndrome is a devastating neurodegenerative disease, typically manifesting in infancy
or early childhood. However, also late-onset cases have been reported. Since its first …

H2S derived from organic thiol metabolism has been proposed serve as an oxygen sensor
in a variety of systems because of its susceptibility to oxidation and its ability to mimic …

Abstract Significance: Although oxygen (O2)-sensing cells and tissues have been known for
decades, the identity of the O2-sensing mechanism has remained elusive. Evidence is …

Mitochondrial diseases are a genetically and phenotypically variable set of monogenic
disorders. The main characteristic of mitochondrial diseases is a defective oxidative …

The ability to detect oxygen availability is a ubiquitous attribute of aerobic organisms.
However, the mechanism (s) that transduce oxygen concentration or availability into …

Eukaryotic cells depend upon oxygen (O 2) for their survival and elaborate mechanisms
have evolved in multicellular animals, especially vertebrates, to monitor the availability of …

BACKGROUND: Short-chain acyl-CoA dehydrogenase deficiency (SCADD) is an autosomal
recessive inborn error of mitochondrial fatty acid oxidation with highly variable biochemical …

Sulfur is a versatile molecule with oxidation states ranging from− 2 to+ 6. From the
beginning, sulfur has been inexorably entwined with the evolution of organisms. Reduced …

Aims: To define the consequences of loss of cysteine dioxygenase (CDO) on cysteine
metabolism at the tissue level, we determined levels of relevant metabolites and enzymes …