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Showing papers in "Biochemical Medicine in 1975"


Journal ArticleDOI
TL;DR: It is speculated, but not proved, that the biochemical abnormality is causally related to the central nervous system dysfunction and compatible with an inherited deficiency of glutaryl-CoA dehydrogenase.

306 citations


Journal ArticleDOI
Kenneth E. McMartin1, A.B. Makar1, G Martin1, M. Palese1, Thomas R. Tephly1 
TL;DR: The use of the monkey as a model for the study of methanol poisoning is presented, and the possible use of 4-methylpyrazole in the treatment of metanol poisoning is implicit.

174 citations


Journal ArticleDOI
TL;DR: In this article, the synthesis and degradation of rat liver metallothionein (MT) were investigated using 14C-cystine as precursor, Cd to stimulate the synthesis of this protein, and Sephadex G-75 chromatography as an isolation method.

156 citations


Journal ArticleDOI
TL;DR: A new method for simultaneous assay of 3,4-dihydroxyphenylalanine, dopamine, norepinephrine, and epinephrine in urine is described, suitable for routine clinical measurement of urinary catecholamines.

154 citations


Journal ArticleDOI
TL;DR: A sensitive and specific assay for formic acid in body fluids has been developed based on the reaction of formate with bacterial formate dehydrogenase coupled to a diaphorase-catalyzed reduction of the nonfluorescent dye resazurin to the fluorescent substance resorufin.

154 citations


Journal ArticleDOI
TL;DR: The data indicate that CDCA suppressed elevated cholesterol and cholestanol synthesis in CTX, and suggest that this primary bile acid may be useful in preventing the clinical progression of this disease.

94 citations


Journal ArticleDOI
TL;DR: An automatic method utilizing ion-exchange column chromatography and fluorescence detection after reaction with orthophthalaldehyde to determine levels of GABA in human CSF, which provides resolution comparable to that of usual amino acid analyses of physiological fluids.

80 citations


Journal ArticleDOI
TL;DR: Addition of an equal volume of 20% polyethylene glycol 6000 to human serum leads to separation of two major zinc ligands, α2-Macroglobulin, a zinc metalloprotein, is precipitated, while albumin, which forms a zinc-protein complex in serum, remains soluble.

78 citations



Journal ArticleDOI
TL;DR: GABA levels were measured in CSF from seven patients with Huntington's chorea and nine control patients with a variety of other neurological disorders as discussed by the authors, and the difference between the two populations was highly significant (P ≤ 0.001).

71 citations


Journal ArticleDOI
TL;DR: The specificity of the semiautomated fluorometric assay technique for homovanillic acid was confirmed by the effects of drugs on the HVA levels in the rat brain and by the distribution of HVA throughout the central nervous system: detectable levels of Hva were only found in the corpus striatum; nucleus accumbens and tuberculum olfactorius.

Journal ArticleDOI
TL;DR: Benzylamine was used as substrate to determine MAO activity in 13 bipolar depression patients, 35 unipolar depressed patients, and 30 controls, and the differences in enzyme activity found in the groups investigated were statistically significant.

Journal ArticleDOI
TL;DR: Analyses of whole blood from a larger group of SS patients showed a wide variation in hematological parameters, in the number of ISC(%), the red cell density distribution, and in hemoglobin composition.

Journal ArticleDOI
TL;DR: The radiometric assay can be used to obtain quantitative information about the behavior of ADA during velocity sedimentation and acrylamide gel electrophoresis and should prove useful in studies of the association of quantitative and qualitative variation in ADA activity with human immunodeficiency diseases.

Journal ArticleDOI
TL;DR: The results showed cell staining by trypan blue to be a relatively poor measure of cell viability and suggest use of the ratio cellular K + Na + or, even more efficiently, the cell content of K+ as sensitive indicators of plasma membrane integrity.

Journal ArticleDOI
TL;DR: This combined HPLC-RIA system offers an efficient and sensitive procedure for separation and estimation of these compounds that is not subject to interference from nonrelated endogenous materials which may affect results obtained with other methods.

Journal ArticleDOI
TL;DR: Since methanol poisoning in the monkey is dependent on its oxidation through alcohol dehydrogenase, 4-methylpyrazole may be a useful adjunct to therapy in the toxicity produced by that alcohol.

Journal ArticleDOI
TL;DR: The urine from two siblings with glutaric aciduria has been found to contain abnormally high amounts of β-hydroxyglutaric and glutaconic acids, which strongly suggests that the enzymic block in these patients is in the decarboxylation of glutaconyl- CoA to crotonyl-CoA.

Journal ArticleDOI
TL;DR: A new, rapid and specific method for the determination of reduced glutathione has been developed and results obtained were consistently lower than those from the DTNB method.

Journal ArticleDOI
TL;DR: The method, when compared to others adopting the same electrode, is recommended for its simplicity, accuracy, and rapidity.

Journal ArticleDOI
TL;DR: The results of these preliminary experiments indicate that TSF can be detected by both a bioassay and an immunoassay in sera of patients with various platelet production disorders.

Journal ArticleDOI
TL;DR: A convenient method for the simultaneous gas chromatographic analysis of endogenous catecholamines (CA) and their alkaloid cyclization products with simple aldehydes (TIQ) which may occur in vivo during alcoholism, has been developed.

Journal ArticleDOI
TL;DR: A procedure for the separation, characterization and detection of picogram quantities of biogenic indolealkylamines contained in cerebrospinal fluid has been described and the unknown amines may be identified by direct comparison with known standards.

Journal ArticleDOI
TL;DR: A composite part of aminophylline, 1,2-diaminoethane, is metabolized in man and rat to N-(2-aminoethyl)acetamide, the structure was assigned by degradation studies and confirmed by synthesis.

Journal ArticleDOI
TL;DR: The clearance ratio for GAG in cases of MPS and ML vary significantly without corresponding changes in the serum level of GAG, suggesting that the glomerular filtrate is not their major source.

Journal ArticleDOI
TL;DR: A rapid spectrophotometric method for the estimation of urinary azathioprine, 6-mercaptopurine, and 6-thiouric acid is described and has been applied successfully to clinical urines.

Journal ArticleDOI
TL;DR: The excretion of 3,4-dihydroxybutyrate was greater than the intake in food in two subjects studied over a long period and the urinary compound had the (S)-configuration, consistent with its originating from the degradation of carbohydrates, though it could also arise from the metabolism of γ-hydroxy butyrate.

Journal ArticleDOI
TL;DR: In well-fed adrenalectomized rats, 5 hours after tryptophan force-feeding, the concentration of plasma glucose and insulin was found to be approximately 50% decreased compared to the initial water-fed control level.

Journal ArticleDOI
TL;DR: The pure α2-macroglobulin obtained was stable for months without change of trypsin binding activity and the excellent correlation of results obtained from the determination of the protein content of the product by four different techniques was reflected.

Journal ArticleDOI
TL;DR: A ribonuclease of MW 33,000 was isolated and purified for normal human urine and obviously is quite different than the pancreatic ribonucleases (MW 13,000) or the ribon nucleases described by other investigators.