Tartratesensitive

Tartrate-sensitive is a term used in medicine, specifically in hematology, to describe a type of enzyme deficiency or anomaly. It refers to a condition where a particular enzyme's activity is significantly affected by the presence of tartrates, which are salts of tartaric acid. The most common context for this term is in relation to the enzyme glutathione reductase (GR).

In some individuals, a genetic variation can lead to a form of glutathione reductase that is unusually sensitive to inhibition by tartrates. This tartrate-sensitive glutathione reductase is still functional, but its activity is reduced when tartrates are present. This sensitivity is often used as a diagnostic marker.

The primary clinical relevance of tartrate-sensitive glutathione reductase is in the diagnosis of a specific, rare form of hereditary hemolytic anemia. Individuals with this condition experience a breakdown of red blood cells, leading to anemia. The anemia is caused by a deficiency in the enzyme glutathione reductase, and the tartrate sensitivity is a key characteristic that helps distinguish this type from other causes of red blood cell fragility.

Diagnostic tests can involve measuring glutathione reductase activity in red blood cells both with and without the addition of tartrates. A significant decrease in enzyme activity in the presence of tartrates is indicative of the tartrate-sensitive form. This finding, combined with clinical symptoms and other laboratory results, aids in establishing the diagnosis and guiding treatment. The specific genetic basis and the precise mechanisms of tartrate inhibition are areas of ongoing research.