Literature DB >> 7564260

Mitochondropathy presenting with non-ketotic hypoglycaemia as 3-hydroxydicarboxylic aciduria.

E Mayatepek1, R J Wanders, M Becker, H J Bremer, G F Hoffmann.   

Abstract

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Year:  1995        PMID： 7564260     DOI： 10.1007/BF00711780

Source DB:  PubMed          Journal:  J Inherit Metab Dis        ISSN： 0141-8955            Impact factor:   4.982


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  11 in total

1.  Studies on the formation of lactate and pyruvate from glucose in cultured skin fibroblasts: implications for detection of respiratory chain defects.

Authors:  F A Wijburg; N Feller; H R Scholte; H Przyrembel; R J Wanders
Journal:  Biochem Int       Date:  1989-09

2.  Marked elevation of urinary 3-hydroxydecanedioic acid in a malnourished infant with glycogen storage disease, mimicking long-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency.

Authors:  J Bergoffen; P Kaplan; D E Hale; M J Bennett; G T Berry
Journal:  J Inherit Metab Dis       Date:  1993       Impact factor: 4.982

3.  Quantitative analysis for organic acids in biological samples: batch isolation followed by gas chromatographic-mass spectrometric analysis.

Authors:  G Hoffmann; S Aramaki; E Blum-Hoffmann; W L Nyhan; L Sweetman
Journal:  Clin Chem       Date:  1989-04       Impact factor: 8.327

4.  Increased excretion of endogenous urinary leukotriene E4 in extrahepatic cholestasis.

Authors:  E Mayatepek; G Pecher
Journal:  Clin Chim Acta       Date:  1993-09-30       Impact factor: 3.786

5.  3-Hydroxyoctanoic aciduria: identification of a new organic acid in the urine of a patient with non-ketotic hypoglycemia.

Authors:  R I Kelley; D H Morton
Journal:  Clin Chim Acta       Date:  1988-06-30       Impact factor: 3.786

6.  3-hydroxydecanedioic acid and related homologues: urinary metabolites in ketoacidosis.

Authors:  J Greter; S Lindstedt; H Seeman; G Steen
Journal:  Clin Chem       Date:  1980-02       Impact factor: 8.327

7.  Urinary 3-hydroxydicarboxylic acids in pathophysiology of metabolic disorders with dicarboxylic aciduria.

Authors:  K Y Tserng; S J Jin; D S Kerr; C L Hoppel
Journal:  Metabolism       Date:  1991-07       Impact factor: 8.694

8.  Fatty acid beta-oxidation in leukocytes from control subjects and medium-chain acyl-CoA dehydrogenase deficient patients.

Authors:  R J Wanders; L Ijlst
Journal:  Biochim Biophys Acta       Date:  1992-01-16

9.  3-Hydroxydicarboxylic aciduria--a fatty acid oxidation defect with severe prognosis.

Authors:  L Hagenfeldt; U von Döbeln; E Holme; J Alm; G Brandberg; E Enocksson; L Lindeberg
Journal:  J Pediatr       Date:  1990-03       Impact factor: 4.406

10.  Secondary 3-hydroxydicarboxylic aciduria mimicking long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency.

Authors:  M J Bennett; M J Weinberger; W G Sherwood; A B Burlina
Journal:  J Inherit Metab Dis       Date:  1994       Impact factor: 4.982
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  8 in total

Review 1.  Compromised fatty acid oxidation in mitochondrial disorders.

Authors:  L Hagenfeldt
Journal:  J Inherit Metab Dis       Date:  1998-08       Impact factor: 4.982

2.  Hypoketonuric 3-hydroxydicarboxylic aciduria in five patients with glycogen storage disease.

Authors:  C E Mize; L J Waber; T Anderson; M J Bennett
Journal:  J Inherit Metab Dis       Date:  1997-07       Impact factor: 4.982

Review 3.  Disorders of mitochondrial fatty acyl-CoA beta-oxidation.

Authors:  R J Wanders; P Vreken; M E den Boer; F A Wijburg; A H van Gennip; L IJlst
Journal:  J Inherit Metab Dis       Date:  1999-06       Impact factor: 4.982

4.  Analysis of 3-hydroxydodecanedioic acid for studies of fatty acid metabolic disorders: preparation of stable isotope standards.

Authors:  J S Chickos; Barbara A Way; J Wilson; M Shaharuzzaman; J Laird; M Landt
Journal:  J Clin Lab Anal       Date:  2002       Impact factor: 2.352

5.  Fatty acid oxidation in fibroblasts from patients with defects in beta-oxidation and in the respiratory chain.

Authors:  N Venizelos; U von Döbeln; L Hagenfeldt
Journal:  J Inherit Metab Dis       Date:  1998-06       Impact factor: 4.982

Review 6.  Disorders of mitochondrial long-chain fatty acid oxidation.

Authors:  R J Pollitt
Journal:  J Inherit Metab Dis       Date:  1995       Impact factor: 4.982

7.  Pyruvate carboxylase deficiency: An underestimated cause of lactic acidosis.

Authors:  F Habarou; A Brassier; M Rio; D Chrétien; S Monnot; V Barbier; R Barouki; J P Bonnefont; N Boddaert; B Chadefaux-Vekemans; L Le Moyec; J Bastin; C Ottolenghi; P de Lonlay
Journal:  Mol Genet Metab Rep       Date:  2014-11-28

8.  Murine deficiency of peroxisomal L-bifunctional protein (EHHADH) causes medium-chain 3-hydroxydicarboxylic aciduria and perturbs hepatic cholesterol homeostasis.

Authors:  Pablo Ranea-Robles; Sara Violante; Carmen Argmann; Tetyana Dodatko; Dipankar Bhattacharya; Hongjie Chen; Chunli Yu; Scott L Friedman; Michelle Puchowicz; Sander M Houten
Journal:  Cell Mol Life Sci       Date:  2021-06-10       Impact factor: 9.207
  8 in total

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