Adrenoleukodystrophy: Elevated C26 fatty acid in cultured skin fibroblasts

Hugo W. Moser; Ann B. Moser; Nariko Kawamura; Jerome Murphy; Kunihiko Suzuki; Herbert Schaumburg; Yasuo Kishimoto

doi:10.1002/ana.410070607

Adrenoleukodystrophy: Elevated C26 fatty acid in cultured skin fibroblasts

Annals of Neurology ◽

10.1002/ana.410070607 ◽

1980 ◽

Vol 7 (6) ◽

pp. 542-549 ◽

Cited By ~ 140

Author(s):

Hugo W. Moser ◽

Ann B. Moser ◽

Nariko Kawamura ◽

Jerome Murphy ◽

Kunihiko Suzuki ◽

...

Keyword(s):

Fatty Acid ◽

Skin Fibroblasts ◽

Cultured Skin

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Adrenoleukodystrophy: Elevated C26 fatty acid in cultured skin fibroblasts and correlation with disease expression in three generations of a kindred

Neurology ◽

10.1212/wnl.32.5.540 ◽

1982 ◽

Vol 32 (5) ◽

pp. 540-540 ◽

Cited By ~ 12

Author(s):

B. P. O'Neill ◽

H. W. Moser ◽

L. C. Marmion

Keyword(s):

Fatty Acid ◽

Skin Fibroblasts ◽

Disease Expression ◽

Three Generations ◽

Cultured Skin

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Glucose-free medium exacerbatesmicrovesicular steatosis in cultured skin fibroblasts of genetic defects of fatty acid oxidation. A novel screening test

Journal of Inherited Metabolic Disease ◽

10.1023/a:1022039206660 ◽

2002 ◽

Vol 25 (7) ◽

pp. 547-555 ◽

Cited By ~ 6

Author(s):

D. L. Renaud ◽

V. Edwards ◽

G. J. Wilson ◽

I. Tein

Keyword(s):

Fatty Acid ◽

Fatty Acid Oxidation ◽

Screening Test ◽

Skin Fibroblasts ◽

Acid Oxidation ◽

Genetic Defects ◽

Free Medium ◽

Cultured Skin

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Diagnosis of Metachromatic Leukodystrophy, Krabbe Disease, and Farber Disease after Uptake of Fatty Acid-labeled Cerebroside Sulfate into Cultured Skin Fibroblasts

Journal of Clinical Investigation ◽

10.1172/jci110607 ◽

1982 ◽

Vol 70 (1) ◽

pp. 89-97 ◽

Cited By ~ 62

Author(s):

Tooru Kudoh ◽

David A. Wenger

Keyword(s):

Fatty Acid ◽

Metachromatic Leukodystrophy ◽

Skin Fibroblasts ◽

Krabbe Disease ◽

Farber Disease ◽

Cerebroside Sulfate ◽

Cultured Skin

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Stimulation of fatty acid uptake and triglyceride synthesis in human cultured skin fibroblasts and adipocytes by a serum protein

Biochemical and Biophysical Research Communications ◽

10.1016/s0006-291x(87)80480-1 ◽

1987 ◽

Vol 144 (1) ◽

pp. 94-100 ◽

Cited By ~ 12

Author(s):

K. Cianflone ◽

P.O. Kwiterovich ◽

M. Walsh ◽

A. Forse ◽

M.A. Rodriguez ◽

...

Keyword(s):

Fatty Acid ◽

Serum Protein ◽

Fatty Acid Uptake ◽

Skin Fibroblasts ◽

Acid Uptake ◽

Triglyceride Synthesis ◽

Cultured Skin ◽

Stimulation Of

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Neonatal multiple acyl-CoA dehydrogenase deficiency: essentially absent fatty acid oxidation activity in proband but normal activity in parental cultured skin fibroblasts

Journal of Inherited Metabolic Disease ◽

10.1007/bf01799277 ◽

1996 ◽

Vol 19 (3) ◽

pp. 379-380 ◽

Cited By ~ 2

Author(s):

W. C. Ip ◽

J. W. Hammond ◽

B. Wilcken

Keyword(s):

Fatty Acid ◽

Fatty Acid Oxidation ◽

Dehydrogenase Deficiency ◽

Normal Activity ◽

Skin Fibroblasts ◽

Acid Oxidation ◽

Oxidation Activity ◽

Cultured Skin

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Effects of exogenous monounsaturated fatty acids on fatty acid metabolism in cultured skin fibroblasts from adrenoleukodystrophy patients

Journal of the Neurological Sciences ◽

10.1016/0022-510x(92)90170-p ◽

1992 ◽

Vol 109 (2) ◽

pp. 207-214 ◽

Cited By ~ 11

Author(s):

Robert Wilson ◽

Douglas R. Tocher ◽

John R. Sargent

Keyword(s):

Fatty Acids ◽

Fatty Acid ◽

Fatty Acid Metabolism ◽

Acid Metabolism ◽

Monounsaturated Fatty Acids ◽

Skin Fibroblasts ◽

Cultured Skin

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Correction of Fatty Acid Oxidation in Carnitine Palmitoyl Transferase 2–Deficient Cultured Skin Fibroblasts by Bezafibrate

Pediatric Research ◽

10.1203/01.pdr.0000083001.91588.bb ◽

2003 ◽

Vol 54 (4) ◽

pp. 446-451 ◽

Cited By ~ 41

Author(s):

Fatima Djouadi ◽

Jean-Paul Bonnefont ◽

Laure Thuillier ◽

Véronique Droin ◽

Noman Khadom ◽

...

Keyword(s):

Fatty Acid ◽

Fatty Acid Oxidation ◽

Skin Fibroblasts ◽

Acid Oxidation ◽

Carnitine Palmitoyl Transferase ◽

Cultured Skin

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Arachidonic fatty acid in red blood cell membranes, lymphocytes, and cultured skin fibroblasts of dominant Charcot-Marie-Tooth syndrome

Journal of the Neurological Sciences ◽

10.1016/0022-510x(93)90273-2 ◽

1993 ◽

Vol 120 (2) ◽

pp. 195-200 ◽

Cited By ~ 2

Author(s):

Lowell L. Williams ◽

Francis S. Wright

Keyword(s):

Fatty Acid ◽

Blood Cell ◽

Red Blood Cell ◽

Cell Membranes ◽

Skin Fibroblasts ◽

Charcot Marie Tooth ◽

Cultured Skin ◽

Red Blood Cell Membranes

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Fatty Acid Metabolism in Cultured Skin Fibroblasts from Patients with Peroxisomal Disorders: Lignoceroyl-CoA Ligase Deficiency in Childhood Adrenoleukodystrophy

Proceedings in Life Sciences - Peroxisomes in Biology and Medicine ◽

10.1007/978-3-642-71325-5_38 ◽

1987 ◽

pp. 358-363 ◽

Cited By ~ 2

Author(s):

M. Hashmi ◽

W. Stanley ◽

I. Singh

Keyword(s):

Fatty Acid ◽

Fatty Acid Metabolism ◽

Acid Metabolism ◽

Skin Fibroblasts ◽

Peroxisomal Disorders ◽

Cultured Skin ◽

Coa Ligase

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Establishing a Reference Interval For Measurement of Flux through the Mitochondrial Fatty Acid Oxidation Pathway in Cultured Skin Fibroblasts

Clinical Chemistry ◽

10.1373/clinchem.2004.045005 ◽

2005 ◽

Vol 51 (3) ◽

pp. 644-646 ◽

Cited By ~ 6

Author(s):

Srinivas B Narayan ◽

Richard L Boriack ◽

Bette Messmer ◽

Michael J Bennett

Keyword(s):

Fatty Acid ◽

Fatty Acid Oxidation ◽

Reference Interval ◽

Skin Fibroblasts ◽

Acid Oxidation ◽

Mitochondrial Fatty Acid Oxidation ◽

Oxidation Pathway ◽

Cultured Skin ◽

Mitochondrial Fatty Acid

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