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Therapeutic Effects of Cystamine in a Murine Model of Huntington's Disease
TLDR
Treatment in R6/2 transgenic HD mice, using the transglutaminase inhibitor cystamine, significantly extended survival, improved body weight and motor performance, and delayed the neuropathological sequela.
Glutathione is present in high concentrations in cultured astrocytes but not in cultured neurons
TLDR
The apparent lack of defense mechanisms against oxidative stress may in part account for the 'fragility' of neurons in culture and the physiological implications of glutathione compartmentation in brain are discussed.
Astrocyte glutamine synthetase: Importance in hyperammonemic syndromes and potential target for therapy
TLDR
The physiology, pathology, and biochemistry of ammonia are reviewed and it is shown how these elements interact to constitute a syndrome that clinicians refer to as hyperammonemic encephalopathy (i.e., acute liver failure, fulminant hepatic failure, chronic liver disease).
A new route for synthesis of dimethylsulphoniopropionate in marine algae
TLDR
In vivo isotope labelling is used to demonstrate that DMSP synthesis in the green macroalga Enteromorpha intestinalis proceeds by a route entirely distinct from that in higher plants, and the fact that a transamination initiates this pathway could help explain how algal DMSP production is enhanced by nitrogen deficiency.
Identification and stereospecificity of the first three enzymes of 3-dimethylsulfoniopropionate biosynthesis in a chlorophyte alga
TLDR
Substrate-specific enzymes catalyzing the first three steps of the DMSP synthesis pathway were detected and partially characterized in cell-free extracts of the chlorophyte alga Enteromorpha intestinalis, and the intermediate DMSHB was shown to act as an osmoprotectant, which indicates that the first four steps may be sufficient to confer osmotolerance.
Central Role of Glutamate Metabolism in the Maintenance of Nitrogen Homeostasis in Normal and Hyperammonemic Brain
TLDR
It is suggested that glutamate may constitute a buffer or bulwark against changes in cerebral amine and ammonia nitrogen, and be placed on the central role of glutamate in the glutamine-glutamate and glutamines-GABA neurotransmitter cycles between neurons and astrocytes.
Inhibition of transglutaminase 2 mitigates transcriptional dysregulation in models of Huntington disease
TLDR
It is demonstrated that selective TG inhibition broadly corrects transcriptional dysregulation in HD and defines a novel HDAC‐independent epigenetic strategy for treating neurodegeneration.
Intranephron distribution of cysteine S -conjugate β-lyase activity and its implication for hexachloro-1,3-butadiene-induced nephrotoxicity in rats
TLDR
The findings suggest that the low Mrβ-lyase is induced by HCBD and that impaired cell function in the segments (especially S3) results in proteins leaking out of the target cells.
Minocycline chelates Ca2+, binds to membranes, and depolarizes mitochondria by formation of Ca2+-dependent ion channels
TLDR
It is shown that minocycline, in the presence of Ca2+, induces selective permeability for small ions, which indirectly prevents induction of the mitochondrial permeability transition.
Treatment of YAC128 mice and their wild‐type littermates with cystamine does not lead to its accumulation in plasma or brain: implications for the treatment of Huntington disease
TLDR
An HPLC system with coulometric detection that can rapidly measure underivatized cystamine, cysteamine and hypotaurine, as well as cysteine and glutathione in the same deproteinized tissue sample is described.
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