PubMed · 5786876
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J G Schoenmakers, J J Gerding, H Bloemendal. 1969. Four different polypeptide chains in alpha-crystallin.. https://pubmed.ncbi.nlm.nih.gov/5786876/
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Liquid chromatography/electrospray ionization-mass spectrometry (LC/ESI-MS) demonstrated that glutathionyl hemoglobin (Hb) levels are increased in patients with diabetes, hyperlipidemia, uremia and Friedreich's ataxia. Glutathionylation of Hb is enhanced by oxidative stress. High performance liquid chromatography (HPLC) and matrix-assisted laser desorption ionization-time of flight mass spectrometry (MALDI-TOF-MS) have also been developed for the quantification of glutathionyl Hb. Glutathionyl-lens proteins were detected in uremic patients and cataractous aged subjects. Glutathionylation of numerous enzymes is induced by oxidative stress, reduces their catalytic activities and may be involved in protection from the damaging effects of oxidative agents. Thioredoxin, glutaredoxin (thioltransferase) and protein disulfide isomerase are the key enzymes in controlling cellular oxidative stress that catalyze reduction of glutathionyl protein disulfide bonds. Thus, protein glutathionylation is closely associated with oxidative stress.
In this paper, the authors reported a new method for measuring transient time-dependent spectra using multi-fiber delay coupler and optical multi-channel analysis technique. The fiber delay coupler consists of five fibers with different lengths and its time-delay between two fibers is determined by the different fiber lengths. The light beams that came from the fiber coupler were coupled by a lens system into the slit of a spectrograph and formed a spectral pattern on the focus plane of the spectrometer. A two-dimensional CCD detector converted the optical pattern into an electrical pattern, and then the transient time-dependent spectra were obtained by a personal computer. The experimental setup was constructed, and the transient time-dependent spectra were measured for KCl crystal shocked by a high speed pill. The time resolution was 20 ns, and the spectral intensity resolution reached 1/18 bit.
We report two patients with motor neuron disease-inclusion dementia, with special reference to the pathology of the motor neuron system and hippocampal formation. The ages of the patients at death were 55 and 62 years, and the disease durations were 8 and 3 years, respectively. The two patients exhibited progressive frontotemporal dementia in the absence of motor neuron signs. At autopsy, both cases exhibited frontotemporal lobar atrophy with ubiquitin-positive, and tau- and alpha-synuclein-negative neuronal inclusions. As expected from the clinical signs, in both cases, the upper and lower motor neuron systems were well preserved: no Bunina bodies or ubiquitinated inclusions were detected in the motor neurons. However, of great importance was that when visualized immunohistochemically, the Golgi apparatus and trans-Golgi network often exhibited fragmentation in the lower motor neurons (the spinal anterior horn cells). In one of the cases, a decrease in the amount of Golgi apparatus was also a frequent feature in the upper motor neurons (Betz cells in the motor cortex). Moreover, in both cases, circumscribed degeneration affecting the CA1-subiculum border zone was evident in the hippocampal formation. These findings further strengthen the idea that, pathologically, motor neuron disease-inclusion dementia is a rare phenotype of amyotrophic lateral sclerosis.