Changes in one-carbon metabolism after duodenal-jejunal bypass surgery.
Dublin, Ireland. In Am J Physiol Endocrinol Metab, Feb 2016
In the liver of DJB rats, betaine-homocysteine S-methyltransferase levels were decreased, whereas serine, cystathionine, cysteine, glutathione, cystathionine beta-synthase, glutathione S-transferase, and aldehyde dehydrogenase levels were increased.
Biosynthesis and Reactivity of Cysteine Persulfides in Signaling.
Ann Arbor, United States. In J Am Chem Soc, Feb 2016
Our detailed kinetic analyses demonstrate a robust capacity for Cys-SSH production by the human transsulfuration pathway enzymes, cystathionine beta-synthase and γ-cystathionase (CSE) and for homocysteine persulfide synthesis from homocystine by CSE only.
Psychiatric manifestations of treatable hereditary metabolic disorders in adults.
Bron, France. In Ann Gen Psychiatry, 2013
This review focuses on three HMD categories: acute, treatable HMDs (urea cycle abnormalities, remethylation disorders, acute intermittent porphyria); chronic, treatable HMDs (Wilson's disease, Niemann-Pick disease type C, homocystinuria due to cystathionine beta-synthase deficiency, cerebrotendinous xanthomatosis); and chronic HMDs that are difficult to treat (lysosomal storage diseases, X-linked adrenoleukodystrophy, creatine deficiency syndrome).
[Hydrogen sulfide and penile erection].
China. In Zhonghua Nan Ke Xue, 2012
In mammalians, H2S is mainly synthesized by two proteases, cystathionine-beta-synthase (CBS) and cystathionine-gamma-lyase (CSE).
Crystal structure of the MgtE Mg2+ transporter.
Yokohama, Japan. In Nature, 2007
The transporter adopts a homodimeric architecture, consisting of the carboxy-terminal five transmembrane domains and the amino-terminal cytosolic domains, which are composed of the superhelical N domain and tandemly repeated cystathionine-beta-synthase domains.
Reduction of false negative results in screening of newborns for homocystinuria.
Boston, United States. In N Engl J Med, 1999
BACKGROUND: Mental retardation and other disabilities (including ectopia lentis, osteoporosis, and thromboembolism) in patients who have homocystinuria as a result of a deficiency of cystathionine beta-synthase can be prevented by the screening of newborns with measurement of blood methionine, followed by the early treatment of affected infants.
Correction of disease-causing CBS mutations in yeast.
Philadelphia, United States. In Nat Genet, 1998
Mutations in cystathionine beta-synthase (CBS) are known to cause homocystinuria, a recessive disorder characterized by excessive levels of total homocysteine (tHcy) in plasma.