The amino-aciduria in Fanconi syndrome. A study making extensive use of techniques based on paper partition chromatography

Dent, C. E.

doi:10.1042/bj0410240

Cited by 415 publications

(61 citation statements)

References 5 publications

(6 reference statements)

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“…and cadmium-ninhydrin (Heathcote & Washington, 1967). Fluorescamine was used according to Mendez & Lai (1975), the Pauly reagent for histidine and tyrosine according to Dent (1947), phenanthrenequinone for arginine according to Yamada & Itano (1966) and proline was detected with isatin.…”

Section: A P a T E R S O N A N D P H C L A R K Ementioning

confidence: 99%

Molecular Basis of Altered Enzyme Specificities in a Family of Mutant Amidases from Pseudomonas aeruginosa

Paterson

Clarke

1979

Journal of General Microbiology

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A family of mutant amidases has been derived by experimental evolution of the aliphatic amidase of Pseudomonas aeruginosa strain PAC I. Mutation amiEI6, in the structural gene for the enzyme, results in the production of the mutant B amidase by strain B6. This strain, unlike the wild-type, can utilize butyramide for growth. Strain B6 gave rise by a single mutational event to strain V9, utilizing valeramide, and strain PhB3, utilizing phenylacetamide. Strain V9 was not itself able to utilize phenylacetamide but gave rise by mutation to the phenylacetamide-utilizing mutant PhVl. Peptide 108 was isolated from chymotryptic digests of mutant amidases from strains B6, PhB3 and PhV1, but could not be detected in chymotryptic digests of the wild-type amidase. The sequence of peptide 108 was established as Met-Arg-His-Gly-Asp-Ile-Phe. Thermolytic digests of mutant amidases from strains B6, PhB3, PhVl and V9 were compared with digests of the wild-type amidase. A peptide of the composition Met, Arg, His, Gly,, Asp,, Ile, Ser,, Thr, Val was found in the digest of the wild-type amidase and was replaced in the digests of the mutant amidases by a peptide of the composition Met, Arg, His, Gly,, Asp,, Ile, Ser,, Thr, Val, Phe. Mutation amiEl6 is common to the four mutant enzymes and can be accounted for by the mutation Ser -+ Phe. The sequence of the chymotryptic peptide corresponds with the N-terminal sequence of the amidase protein, and can also be related to the thermolysin peptides. It is concluded that mutation amiEl6 is a Ser -+ Phe change at position 7 from the N-terminus and the effect of this on the enzyme conformation is discussed.

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Section: A P a T E R S O N A N D P H C L A R K Ementioning

confidence: 99%

Molecular Basis of Altered Enzyme Specificities in a Family of Mutant Amidases from Pseudomonas aeruginosa

Paterson

Clarke

1979

Journal of General Microbiology

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“…The positions of the separated sugars on guide strips were revealed by the spray reagent and the corresponding areas excised from the experimental and blank chromatograms. Sugar and corresponding blank strips were quantitatively eluted with distilled water (Dent, 1947) and collected into measuring cylinders (5 ml.) which stood in an ice-bath and contained 0.4 % (w/v) I-naphthol in concentrated sulphuric acid (2.5 ml.…”

Section: Analysis Of Bacteriocin Bacteriocin Solutionmentioning

confidence: 99%

Properties of a Proteus morganii Bacteriocin

Smit¹,

Klerk

Coetzee

1968

Journal of General Microbiology

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SUMMARYThe bacteriocin produced by Proteus morganii strain MR 336 is a thermolabile glycoprotein with a sedimentation constant of 4 0 . Activity is dependent on the integrity of the complex and the bacteriocin is inactivated by oxidation. Bacteriocinogeny could not be transmitted to other strains of P. morgunii but was eliminated by 30,ug./ml. acridine orange at pH 7.45. Bacteriocin-resistant mutants of a sensitive P. morganii strain were readily obtained.

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“…The same type of symptom complex, but presenting as a severe osteomalacia in adult life, has also been recognized (Milkman, 1930; Stowers & Dent, 1947). In such adult cases cystinosis has not yet been found, although gross aminoaciduria occurred in one of these cases (Dent, 1947).…”

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confidence: 99%

“…It should be noted that, in these cases, the level of plasma amino-nitrogen was not markedly raised and the pattern of plasma amino-acids was not abnormal. This aminoaciduria has also been interpreted therefore as being of renal origin (Dent, 1947) and the result of defective tubular reabsorption. A lowered renal threshold for glucose was also present in these cases, and it was suggested that incomplete tubular reabsorption of phosphate also occurred and was the cause of the hypophosphataemia and the bony lesions (Dent, 1947).…”

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confidence: 99%

The Genetics of ‘Cystinuria’

Dent

Harris

1951

Annals of Eugenics

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Cystine is a normal constituent of human urine. Under ordinary conditions, healthy individuals excrete mg. of this substance in the free state daily (Reed, 1942). In the urine of certain people, however, much larger quantities of cystine are regularly found, and it is to this condition that the term cystinuria has come to be applied. The disorder was first investigated by Wollaston in 1810 as the result of the discovery of a bladder-stone of unusual composition, and, until the work of Lewis in 1932, it had always been regarded as a rare anomaly. It was thought that most cases suffered a greater or lesser degree of disability, leading eventually to renal failure. This was sometimes attributed to stone formation, sometimes to direct damage to the kidney by the large quantities of cystine in the process of excretion. However, symptomless cases, usually among the close relatives of patients with cystine calculi, had occasionally been recognized. Such individuals were identified by the finding of cystine crystals in their urine.Lewis (1932) threw a completely new light on the situation. By using more reliable chemical tests for cystine, he was able to demonstrate that gross cystinuria could occur in individuals without either the appearance of cystine crystals in the urine or of cystine stone formation, and he showed that, far from being a rare anomaly, the condition was relatively common. He made a systematic examination of the urines of 10,534 students. Besides ordinary microscopic examination for cystine crystals, he also applied the Sullivan and the cyanide-nitroprusside tests for cystine. He found, in all, eighteen individuals who gave strong positive reactions by both these tests, and in only four of these could cystine crystals be demonstrated in the urinary deposit. These eighteen subjects were probably excreting between 200 and 1000 mg. of cystine daily, and repeated examination showed that the phenomenon was constantly present. In none of them was there any history of the occurrence of renal calculi, and in other respects they appeared to be perfectly well developed and healthy. Thus approximately 1 in 600 of the population studied were excreting cystine in quantities greatly in excess of that usually found. I n addition to these eighteen people, another eleven individuals gave weak positive tests for cystine and another eleven were observed who gave occasional weak positives.

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The amino-aciduria in Fanconi syndrome. A study making extensive use of techniques based on paper partition chromatography

Cited by 415 publications

References 5 publications

Molecular Basis of Altered Enzyme Specificities in a Family of Mutant Amidases from Pseudomonas aeruginosa

Molecular Basis of Altered Enzyme Specificities in a Family of Mutant Amidases from Pseudomonas aeruginosa

Properties of a Proteus morganii Bacteriocin

The Genetics of ‘Cystinuria’

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