Literature DB >> 7990664

Serum fatty acid profiles in cystic fibrosis patients and their parents.

A B Christophe1, W J Warwick, R T Holman.   

Abstract

Fatty acid compositions of the major serum lipid classes from 43 cystic fibrosis (CF) homozygotes (CF patients), 36 obligate heterozygotes (parents of CF patients) and 34 controls were determined by capillary gas chromatography. Fatty acid compositions of the homozygote CF patients were skewed in the direction of relative essential fatty acid deficiency in comparison with the controls. Less pronounced, but similar deviations from normal, were observed in the heterozygotes. Homozygotes with normal fatty acid compositions and heterozygotes with considerably disturbed fatty acid profiles were found.

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Year:  1994        PMID： 7990664     DOI： 10.1007/BF02536629

Source DB:  PubMed          Journal:  Lipids        ISSN： 0024-4201            Impact factor:   1.880


  37 in total

1.  A therapeutic trial of fatty acid supplementation in cystic fibrosis.

Authors:  R B Elliott
Journal:  Pediatrics       Date:  1976-04       Impact factor: 7.124

2.  Effect on renal function of essential fatty acid supplementation in cystic fibrosis.

Authors:  B Strandvik; U Berg; A Kallner; E Kusoffsky
Journal:  J Pediatr       Date:  1989-08       Impact factor: 4.406

3.  Effect of sex and age on fatty acid composition of human serum lipids.

Authors:  R T Holman; L Smythe; S Johnson
Journal:  Am J Clin Nutr       Date:  1979-12       Impact factor: 7.045

4.  Deficiencies of polyunsaturated fatty acids and replacement by nonessential fatty acids in plasma lipids in multiple sclerosis.

Authors:  R T Holman; S B Johnson; E Kokmen
Journal:  Proc Natl Acad Sci U S A       Date:  1989-06       Impact factor: 11.205

5.  Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA.

Authors:  J R Riordan; J M Rommens; B Kerem; N Alon; R Rozmahel; Z Grzelczak; J Zielenski; S Lok; N Plavsic; J L Chou
Journal:  Science       Date:  1989-09-08       Impact factor: 47.728

6.  Essential fatty acid status in cystic fibrosis and the effects of safflower oil supplementation.

Authors:  J D Lloyd-Still; S B Johnson; R T Holman
Journal:  Am J Clin Nutr       Date:  1981-01       Impact factor: 7.045

7.  PREPARATION OF FATTY ACID METHYL ESTERS AND DIMETHYLACETALS FROM LIPIDS WITH BORON FLUORIDE--METHANOL.

Authors:  W R MORRISON; L M SMITH
Journal:  J Lipid Res       Date:  1964-10       Impact factor: 5.922

8.  Essential fatty acid deficiency in malnourished children.

Authors:  R T Holman; S B Johnson; O Mercuri; H J Itarte; M A Rodrigo; M E De Tomas
Journal:  Am J Clin Nutr       Date:  1981-08       Impact factor: 7.045

9.  Identification of the cystic fibrosis gene: chromosome walking and jumping.

Authors:  J M Rommens; M C Iannuzzi; B Kerem; M L Drumm; G Melmer; M Dean; R Rozmahel; J L Cole; D Kennedy; N Hidaka
Journal:  Science       Date:  1989-09-08       Impact factor: 47.728

10.  Relationship of decreased hepatic lipase activity and lipoprotein abnormalities to essential fatty acid deficiency in cystic fibrosis patients.

Authors:  E Lévy; G Lepage; M Bendayan; N Ronco; L Thibault; N Galéano; L Smith; C C Roy
Journal:  J Lipid Res       Date:  1989-08       Impact factor: 5.922
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  10 in total

1.  Linoleic acid supplement in cystic fibrosis: friend or foe?

Authors:  Neeraj Vij
Journal:  Am J Physiol Lung Cell Mol Physiol       Date:  2010-08-13       Impact factor: 5.464

2.  Low linoleic and high docosahexaenoic acids in a severe phenotype of transgenic cystic fibrosis mice.

Authors:  Birgitta Strandvik; Wanda K O Neal; Mohamed A Ali; Ulf Hammar
Journal:  Exp Biol Med (Maywood)       Date:  2018-03

3.  Renal function in pediatric cystic fibrosis patients in the first decade of life.

Authors:  Chanel Prestidge; Mark A Chilvers; A George F Davidson; Eva Cho; Vanessa McMahon; Colin T White
Journal:  Pediatr Nephrol       Date:  2010-12-29       Impact factor: 3.714

Review 4.  Role for animal models in understanding essential fatty acid deficiency in cystic fibrosis.

Authors:  Deanne H Hryciw; Courtney A Jackson; Nirajan Shrestha; David Parsons; Martin Donnelley; Andrew J McAinch
Journal:  Cell Mol Life Sci       Date:  2021-11-05       Impact factor: 9.261

5.  A mechanism accounting for the low cellular level of linoleic acid in cystic fibrosis and its reversal by DHA.

Authors:  M Rabie Al-Turkmani; Charlotte Andersson; Ragheed Alturkmani; Waddah Katrangi; Joanne E Cluette-Brown; Steven D Freedman; Michael Laposata
Journal:  J Lipid Res       Date:  2008-05-14       Impact factor: 5.922

6.  DeltaF508 CFTR Hetero- and Homozygous Paediatric Patients with Cystic Fibrosis Do Not Differ with Regard to Nutritional Status.

Authors:  Aleksandra Mędza; Katarzyna Kaźmierska; Bartosz Wielgomas; Lucyna Konieczna; Ilona Olędzka; Agnieszka Szlagatys-Sidorkiewicz; Katarzyna Sznurkowska
Journal:  Nutrients       Date:  2021-04-21       Impact factor: 5.717

7.  Determinants of Serum Glycerophospholipid Fatty Acids in Cystic Fibrosis.

Authors:  Sławomira Drzymała-Czyż; Patrycja Krzyżanowska; Berthold Koletzko; Jan Nowak; Anna Miśkiewicz-Chotnicka; Jerzy A Moczko; Aleksandra Lisowska; Jarosław Walkowiak
Journal:  Int J Mol Sci       Date:  2017-01-18       Impact factor: 5.923

8.  A novel lipidomic strategy reveals plasma phospholipid signatures associated with respiratory disease severity in cystic fibrosis patients.

Authors:  Ida Chiara Guerrera; Giuseppe Astarita; Jean-Philippe Jais; Dorota Sands; Anna Nowakowska; Julien Colas; Isabelle Sermet-Gaudelus; Martin Schuerenberg; Daniele Piomelli; Aleksander Edelman; Mario Ollero
Journal:  PLoS One       Date:  2009-11-06       Impact factor: 3.240

Review 9.  Abnormal unsaturated fatty acid metabolism in cystic fibrosis: biochemical mechanisms and clinical implications.

Authors:  Adam C Seegmiller
Journal:  Int J Mol Sci       Date:  2014-09-11       Impact factor: 5.923

Review 10.  Is the ENaC Dysregulation in CF an Effect of Protein-Lipid Interaction in the Membranes?

Authors:  Birgitta Strandvik
Journal:  Int J Mol Sci       Date:  2021-03-08       Impact factor: 5.923
  10 in total

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