Usage Information

Familial hyperlysinemia with lysine-ketoglutarate reductase insufficiency
Joseph Dancis, … , Rody P. Cox, Norman C. Woody
Joseph Dancis, … , Rody P. Cox, Norman C. Woody
Published August 1, 1969
Citation Information: J Clin Invest. 1969;48(8):1447-1452. https://doi.org/10.1172/JCI106110.
View: Text | PDF
Research Article

Familial hyperlysinemia with lysine-ketoglutarate reductase insufficiency

Abstract

Fibroblasts grown in tissue culture from the skin of normal subjects have lysine-ketoglutarate reductase activity (lysine: α-ketoglutarate: triphosphopyridine nucleotide (TPNH) oxidoreductase (ε-N-[L-glutaryl-2]-L-lysine forming)). The activity of the enzyme is considerably reduced in the skin fibroblasts grown from three siblings with hyperlysinemia. The high concentrations of lysine in the blood of these patients, the previous demonstration in the intact subject of a reduction in the ability to degrade lysine, and the present demonstration of diminished lysine-ketoglutarate reductase activity, accurately define the metabolic defect and establish the saccharopine (ε-N-[L-glutaryl-2]-L-lysine) pathway as the major degradative pathway for lysine in the human.

Authors

Joseph Dancis, Joel Hutzler, Rody P. Cox, Norman C. Woody

×

Usage data is cumulative from August 2024 through August 2025.

Usage JCI PMC
Text version 158 13
PDF 64 13
Scanned page 216 6
Citation downloads 98 0
Totals 536 32
Total Views 568
(Click and drag on plot area to zoom in. Click legend items above to toggle)

Usage information is collected from two different sources: this site (JCI) and Pubmed Central (PMC). JCI information (compiled daily) shows human readership based on methods we employ to screen out robotic usage. PMC information (aggregated monthly) is also similarly screened of robotic usage.

Various methods are used to distinguish robotic usage. For example, Google automatically scans articles to add to its search index and identifies itself as robotic; other services might not clearly identify themselves as robotic, or they are new or unknown as robotic. Because this activity can be misinterpreted as human readership, data may be re-processed periodically to reflect an improved understanding of robotic activity. Because of these factors, readers should consider usage information illustrative but subject to change.

Advertisement