3-Hydroxy-3-methylglutaryl-CoA lyase in human skin fibroblasts: study of its properties and deficient activity in 3-hydroxy-3-methylglutaric aciduria patients using a simple spectrophotometric method

R. J. Wanders; R. B. Schutgens; P. H. Zoeters

doi:https://doi.org/10.1016/0009-8981(88)90294-X

3-Hydroxy-3-methylglutaryl-CoA lyase in human skin fibroblasts: study of its properties and deficient activity in 3-hydroxy-3-methylglutaric aciduria patients using a simple spectrophotometric method

R. J. Wanders, R. B. Schutgens, P. H. Zoeters

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Research output: Contribution to journal › Article › Academic › peer-review

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Abstract

In this paper we studied the properties of 3-hydroxy-3-methylglutaryl-CoA lyase (HMG-CoA lyase) in human skin fibroblasts. The enzyme was found to exhibit an absolute requirement for divalent cations such as magnesium. Furthermore, dithiothreitol was necessary for full activity. The enzyme was found to be maximally active at pH 9.25. When measured at this pH in the presence of magnesium and dithiothreitol enzyme activity was high enough to be determined by simple spectrophotometry by measuring the amounts of acetoacetate produced. The results obtained suggest that the large variation in the values reported in literature for the activity of HMG-CoA lyase in human skin fibroblasts is due to the fact that the enzyme shows little activity at pH values below 8

Original language	English
Pages (from-to)	95-101
Journal	Clinica chimica acta; international journal of clinical chemistry
Volume	171
Issue number	1
DOIs	https://doi.org/10.1016/0009-8981(88)90294-X
Publication status	Published - 1988

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https://doi.org/10.1016/0009-8981(88)90294-X

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Wanders, R. J., Schutgens, R. B., & Zoeters, P. H. (1988). 3-Hydroxy-3-methylglutaryl-CoA lyase in human skin fibroblasts: study of its properties and deficient activity in 3-hydroxy-3-methylglutaric aciduria patients using a simple spectrophotometric method. Clinica chimica acta; international journal of clinical chemistry, 171(1), 95-101. https://doi.org/10.1016/0009-8981(88)90294-X

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title = "3-Hydroxy-3-methylglutaryl-CoA lyase in human skin fibroblasts: study of its properties and deficient activity in 3-hydroxy-3-methylglutaric aciduria patients using a simple spectrophotometric method",

abstract = "In this paper we studied the properties of 3-hydroxy-3-methylglutaryl-CoA lyase (HMG-CoA lyase) in human skin fibroblasts. The enzyme was found to exhibit an absolute requirement for divalent cations such as magnesium. Furthermore, dithiothreitol was necessary for full activity. The enzyme was found to be maximally active at pH 9.25. When measured at this pH in the presence of magnesium and dithiothreitol enzyme activity was high enough to be determined by simple spectrophotometry by measuring the amounts of acetoacetate produced. The results obtained suggest that the large variation in the values reported in literature for the activity of HMG-CoA lyase in human skin fibroblasts is due to the fact that the enzyme shows little activity at pH values below 8",

author = "Wanders, {R. J.} and Schutgens, {R. B.} and Zoeters, {P. H.}",

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Wanders, RJ, Schutgens, RB & Zoeters, PH 1988, '3-Hydroxy-3-methylglutaryl-CoA lyase in human skin fibroblasts: study of its properties and deficient activity in 3-hydroxy-3-methylglutaric aciduria patients using a simple spectrophotometric method', Clinica chimica acta; international journal of clinical chemistry, vol. 171, no. 1, pp. 95-101. https://doi.org/10.1016/0009-8981(88)90294-X

3-Hydroxy-3-methylglutaryl-CoA lyase in human skin fibroblasts: study of its properties and deficient activity in 3-hydroxy-3-methylglutaric aciduria patients using a simple spectrophotometric method. / Wanders, R. J.; Schutgens, R. B.; Zoeters, P. H.
In: Clinica chimica acta; international journal of clinical chemistry, Vol. 171, No. 1, 1988, p. 95-101.

Research output: Contribution to journal › Article › Academic › peer-review

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Wanders RJ, Schutgens RB, Zoeters PH. 3-Hydroxy-3-methylglutaryl-CoA lyase in human skin fibroblasts: study of its properties and deficient activity in 3-hydroxy-3-methylglutaric aciduria patients using a simple spectrophotometric method. Clinica chimica acta; international journal of clinical chemistry. 1988;171(1):95-101. doi: https://doi.org/10.1016/0009-8981(88)90294-X