Acid lipase activity of human lymphocytes

Coates, Paul M.; Cortner, Jean A.; Hoffman, George M.; Brown, Spencer A.

doi:10.1016/0005-2760(79)90038-9

Cited by 34 publications

(9 citation statements)

References 14 publications

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“…The enzyme has been found in aortic tissue of several mammalian species, including humans and has been described recently in rabbit brain microvessels (Brecher and Kuan, 1979); variations in activity with vessel site have not yet been reported. In addition, an ACE has been found in macrophages (Werb and Cohn, 1972), liver (Nilsson et al, 1973), lymphocytes (Coates et al, 1979), and kidney (Wolinsky, H., unpublished observations).…”

Section: Lysosomal Lipases and Catabolism Of Ldlmentioning

confidence: 94%

A proposal linking clearance of circulating lipoproteins to tissue metabolic activity as a basis for understanding atherogenesis.

Wolinsky¹

1980

Circulation Research

141

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Section: Lysosomal Lipases and Catabolism Of Ldlmentioning

confidence: 94%

A proposal linking clearance of circulating lipoproteins to tissue metabolic activity as a basis for understanding atherogenesis.

Wolinsky¹

1980

Circulation Research

141

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“…Mononuclear leukocytes from patients R-1, J-1, and their parents were isolated from 8 to 30 ml of chelated blood as previously described by Coates et al (9). Contaminating erythrocytes were removed by hypotonic lysis.…”

Section: Methodsmentioning

confidence: 99%

Long-Chain Acyl Coenzyme A Dehydrogenase Deficiency: An Inherited Cause of Nonketotic Hypoglycemia

et al. 1985

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ABSTRACT. Three children from unrelated families presented in early childhood with hypoglycemia and cardiorespiratory arrests associated with fasting. Significant hepatomegaly, cardiomegaly, and hypotonia were present at the time of initial presentation. Ketones were not present in the urine at the time of hypoglycemia in any patient; however, dicarboxylic aciduria was documented in one patient at the time of the acute episode and in two patients during fasting studies. Total plasma carnitine concentration was low with an increased esterified carnitine fraction. These findings suggested a defect in mitochondria1 fatty acid oxidation, and specific assays were performed for the acyl coenzyme A (CoA) dehydrogenases. These analyses showed that the activity of the long-chain acyl CoA dehydrogenase was less than 10% of control values in fibroblasts, leukocytes, and liver tissue. Activities of the medium-chain, short-chain, and isovaleryl CoA dehydrogenases were not different from control values. With cultured fibroblasts, COz evolution from long-chain fatty acids was significantly reduced, while COz evolution from mediumchain and short-chain fatty acids was comparable to control values-findings consistent with a defect early in the /3- Vol. 19, No. 7, 1985 Printed in U.S.A.oxidation sequence. Studies of acyl CoA dehydrogenase activities in fibroblasts and leukocytes from parents of the patients showed levels of long-chain acyl CoA dehydrogenase activity intermediate between affected and control values and indicated an autosomal recessive form of inheritance of this enzymatic defect. These results describe a previously unrecognized metabolic disorder of fatty acid oxidation due to a deficiency of the long-chain acyl CoA dehydrogenase which may present in early childhood with disastrous consequences. This diagnosis should be considered in children who present with nonketotic hypoglycemia, carnitine insufficiency, and inadequately explained cardiorespiratory arrests. (Pediatr Res 19: 666-671, 1985) Abbreviations CoA, coenzyme A ETF, electron transfer flavoprotein

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“…Mononuclear leukocytes were isolated from six patients (F-1, S-1, W-1, Z-1, and patients 1 3 and 14), seven of their available parents (F-2, F-3, S-2, S-3, W-2, 2-2, Z-3), and one unaffected sibling (2-4) by the method previously described by Coates et al (9). Briefly, heparinized blood was diluted 1:l with Hanks' balanced salt solution (pH 7.2) and layered over Ficoll-Paque (Pharmacia) in siliconized glass centrifuge tubes.…”

Section: Methodsmentioning

confidence: 99%

Genetic Deficiency of Medium-Chain Acyl Coenzyme A Dehydrogenase: Studies in Cultured Skin Fibroblasts and Peripheral Mononuclear Leukocytes

et al. 1985

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Acid lipase activity of human lymphocytes

Cited by 34 publications

References 14 publications

A proposal linking clearance of circulating lipoproteins to tissue metabolic activity as a basis for understanding atherogenesis.

A proposal linking clearance of circulating lipoproteins to tissue metabolic activity as a basis for understanding atherogenesis.

Long-Chain Acyl Coenzyme A Dehydrogenase Deficiency: An Inherited Cause of Nonketotic Hypoglycemia

Genetic Deficiency of Medium-Chain Acyl Coenzyme A Dehydrogenase: Studies in Cultured Skin Fibroblasts and Peripheral Mononuclear Leukocytes

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