Metabolism of sulfur-containing amino acids in a patient excreting β-mercaptolactate-cysteine disulfide

Crawhall, J. C.; Purkiss, P.; Stanbury, John B.

doi:10.1016/0006-2944(73)90105-1

Cited by 8 publications

(7 citation statements)

References 5 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Human MCDU was reported to be associated with behavioral abnormalities (mental retardation891011121315, hypokinetic behavior8, and grand mal seizures8) and anomalies (flattened nasal bridge8 and excessively arched palate8); however, the pathogenesis has not been clarified since MCDU was recognized more than 40 years ago. Macroscopic anomalies were associated in 1 case8; however, this could be an accidental combination.…”

Section: Discussionmentioning

confidence: 99%

“…As an alternative functional diversity of MST, we recently demonstrated in vitro that MST can produce sulfur oxides (SO x ) in the redox cycle of persulfide (-S-S − ) formed at the catalytic-site cysteine of the reaction intermediate7. To elucidate these functional diversity of MST in living organism, we tried to generate homogynous (null) MST-knockout (MST-KO) mouse which was a animal model of mercaptolactate-cysteine disulfiduria (MCDU)8910111213141516. MCDU was first recognized and reported in 19688 as an inherited metabolic disorder caused by congenital MST insufficiency or deficiency.…”

mentioning

confidence: 99%

“…MCDU was first recognized and reported in 19688 as an inherited metabolic disorder caused by congenital MST insufficiency or deficiency. Most cases were associated with mental retardation891011121315, while the pathogenesis remains unknown. In the present study, we revealed by means of comprehensive pathologic, morphologic and functional analyses that MST-KO mice, these mice showed increased anxiety-like behaviors as higher brain dysfunction associated with increase in serotonin level in the brain.…”

mentioning

confidence: 99%

See 2 more Smart Citations

Antioxidant enzyme, 3-mercaptopyruvate sulfurtransferase-knockout mice exhibit increased anxiety-like behaviors: a model for human mercaptolactate-cysteine disulfiduria

Nagahara

Nagano

Ito

et al. 2013

Sci Rep

View full text Add to dashboard Cite

Human mercaptolactate-cysteine disulfiduria (MCDU) was first recognized and reported in 1968. Most cases of MCDU are associated with mental retardation, while the pathogenesis remains unknown. To investigate it, we generated homozygous 3-mercaptopyruvate sulfurtransferase (MST: EC 2.8.1.2) knockout (KO) mice using C57BL/6 embryonic stem cells as an animal model. The MST-KO mice showed significantly increased anxiety-like behaviors with an increase in serotonin level in the prefrontal cortex (PFC), but not with abnormal morphological changes in the brain. MCDU can be caused by loss in the functional diversity of MST; first, MST functions as an antioxidant protein. MST possessing 2 redox-sensing molecular switches maintains cellular redox homeostasis. Second, MST can produce H2S (or HS−). Third, MST can also produce SOx. It is concluded that behavioral abnormality in MST-KO mice is caused by MST function defects such as an antioxidant insufficiency or a new transducer, H2S (or HS−) and/or SOx deficiency.

show abstract

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

See 1 more Smart Citation

Antioxidant enzyme, 3-mercaptopyruvate sulfurtransferase-knockout mice exhibit increased anxiety-like behaviors: a model for human mercaptolactate-cysteine disulfiduria

Nagahara

Nagano

Ito

et al. 2013

Sci Rep

View full text Add to dashboard Cite

show abstract

“…Oral administration of L-cysteine increased this excretion but L-methioninc by the same route had very little effect (Crawhall, Bir, Purkiss and Stanbury, 1971). Intravenous administration oi L['*C] cystine caused the rapid appearance of radioactive p MLCD in the urine whereas intravenous L[^^S] metliionine produced no labelled ^ MLCD in the urine (Crawhall, Purkiss and Stanbury, 1973). No ^ MLCD could be detected in his plasma.…”

Section: Discussionmentioning

confidence: 99%

Beta‐mercaptolactate Cysteine Disulphiduria in a Mentally Retarded Scottish Male

Law

Fowler

1976

J intellect Disabil Res

View full text Add to dashboard Cite

“…It is anatomically located in proximal tubule epithelium, pericentral hepatocytes, cardiac cells and brain neuroglial cells [204]. In a human study, it was found that approximately 15% of the total cysteine was metabolized by the MST pathway [205]. Rat has higher levels of this pathway than guinea pig [200].…”

Section: H 2 S Biosynthesismentioning

confidence: 98%

Interactive Endogenous Small Molecule (Gaseous) Signaling: Implications for Teratogenesis

Fukuto¹,

Collins²

2007

CPD

View full text Add to dashboard Cite

Dioxygen (O2) is an exogenously supplied gas with a number of properties that make it valuable as a biological source of energy and as a result much of life has become dependent on this molecule. Nitric oxide (NO), carbon dioxide (CO) and hydrogen sulfide (H2S) are small molecules that are sometimes in a gaseous state and that can be either exogenously or endogenously supplied. The chemistry of these four molecules allows them to share some common biological targets and signal transduction pathways as well as providing for unique aspects to the biochemistry of each one. Dioxygen can be teratogenic either in excess (hyperoxia) or in deficiency (hypoxia). Although there is a great deal known about the chemistry and physiology of dioxygen, the mechanisms by which it induces toxic endpoints, such as teratogenesis, are unknown. This review examines some fundamental concepts of these four signaling molecules and considers some of the molecular targets and pathways by which they interact. The information regarding the teratogenicity of either excess or deficiency of the four gases is summarized. Interaction information is generally unavailable for teratogenicity endpoints with the four gases and also a mechanistic understanding of the toxicodynamics of the compounds is lacking. Although it could be theoretically predicted that certain interactions would be additive, for example carbon monoxide and hypoxia, based on the physiological role of these molecules, the data is unavailable. Consequently, these small (gaseous) signaling molecules have been demonstrated to interact with respect to signaling pathways, but whether this indicates a similar result for teratogenesis remains unevaluated.

show abstract

Metabolism of sulfur-containing amino acids in a patient excreting β-mercaptolactate-cysteine disulfide

Cited by 8 publications

References 5 publications

Antioxidant enzyme, 3-mercaptopyruvate sulfurtransferase-knockout mice exhibit increased anxiety-like behaviors: a model for human mercaptolactate-cysteine disulfiduria

Antioxidant enzyme, 3-mercaptopyruvate sulfurtransferase-knockout mice exhibit increased anxiety-like behaviors: a model for human mercaptolactate-cysteine disulfiduria

Beta‐mercaptolactate Cysteine Disulphiduria in a Mentally Retarded Scottish Male

Interactive Endogenous Small Molecule (Gaseous) Signaling: Implications for Teratogenesis

Contact Info

Product

Resources

About