English Journal

Neutral aminoaciduria in cystathionine β-synthase-deficient mice; an animal model of homocystinuria.

Akahoshi N1, Kamata S2, Kubota M2, Hishiki T3, Nagahata Y3, Matsuura T3, Yamazaki C4, Yoshida Y4, Yamada H4, Ishizaki Y4, Suematsu M3, Kasahara T2, Ishii I5.
American journal of physiology. Renal physiology.Am J Physiol Renal Physiol.2014 Jun 15;306(12):F1462-76. doi: 10.1152/ajprenal.00623.2013. Epub 2014 Apr 23.
The kidney is one of the major loci for the expression of cystathionine β-synthase (CBS) and cystathionine γ-lyase (CTH). While CBS-deficient (Cbs(-/-)) mice display homocysteinemia/methioninemia and severe growth retardation, and rarely survive beyond the first 4 wk, CTH-deficient (Cth(-/-)) mice
PMID 24761004

Cystathionine gamma-Lyase-deficient mice require dietary cysteine to protect against acute lethal myopathy and oxidative injury.

Ishii I1, Akahoshi N, Yamada H, Nakano S, Izumi T, Suematsu M.
The Journal of biological chemistry.J Biol Chem.2010 Aug 20;285(34):26358-68. doi: 10.1074/jbc.M110.147439. Epub 2010 Jun 21.
Cysteine is considered a nonessential amino acid in mammals as it is synthesized from methionine via trans-sulfuration. However, premature infants or patients with hepatic failure may require dietary cysteine due to a lack of cystathionine gamma-lyase (CTH), a key trans-sulfuration enzyme. Here, we
PMID 20566639

Related Pictures

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