Cells have a constant turnover of proteins that recycle most amino acids over time. Net loss
is mainly due to amino acid oxidation. Homeostasis is achieved through exchange of …

The transport of amino acids in kidney and intestine is critical for the supply of amino acids to
all tissues and the homeostasis of plasma amino acid levels. This is illustrated by a number …

The small intestine mediates the absorption of amino acids after ingestion of protein and
sustains the supply of amino acids to all tissues. The small intestine is an important …

Macrophage activation syndrome (MAS), or secondary hemophagocytic lymphohistiocytosis
(HLH), is a cytokine storm syndrome associated with multi-organ system dysfunction and …

Amino acids are essential building blocks of all mammalian cells. In addition to their role in
protein synthesis, amino acids play an important role as energy fuels, precursors for a …

Oral supplements of arginine and citrulline increase local nitric oxide (NO) production in the
small intestine and this may be harmful under certain circumstances. Gastrointestinal toxicity …

An increased concentration of ammonia is a non-specific laboratory sign indicating the
presence of potentially toxic free ammonia that is not normally removed. This does occur in …

Background Lysinuric protein intolerance (LPI) is a rare metabolic disease resulting from
recessive-inherited mutations in the SLC7A7 gene encoding the cationic amino-acids …

Heteromeric amino acid transporters (HATs) are composed of a heavy (SLC3 family) and a
light (SLC7 family) subunit. Mutations in system b0,+(rBAT-b0,+ AT) and in system y+ L …

Background Hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome is a
rare autosomal recessive disorder of the urea cycle. HHH has a panethnic distribution, with …