نتایج جستجو برای: spacity very long chain fatty acid

تعداد نتایج: 2315483  

A. Pikhtirova, A. Zachwieja E. Pecka-Kiełb F. Zigo J. Bujok J. Elečko M. Vasil

Background: Mastitis in sheep caused by Staphylococcus spp. is a serious concern for dairy farming. Aims: The objective of this study was to evaluate the impact of the intramammary infection (IMI) caused by Staphylococcus spp. on the long-chain fatty acid profile and composition of ewe’s milk. Methods: The experiment was conducted in a herd of Zoslachtena Valaska sheep. Half-udder milk samples ...

Journal: :Human molecular genetics 1998
L T Braiterman S Zheng P A Watkins M T Geraghty G Johnson M C McGuinness A B Moser K D Smith

X-Linked adrenoleukodystrophy (X-ALD) is a neurodegenerative disorder characterized by reduced peroxisomal very long chain fatty acid (VLCFA) beta-oxidation. The X - ALD gene product (ALDP) is a peroxisomal transmembrane protein with an ATP binding cassette (ABC). ALDP and three other ABC proteins (PMP70, ALDR, P70R) localize to the peroxisomal membrane. The function of this family of peroxisom...

Journal: :Obstetrics and gynecology 2006
Marsha F Browning Harvey L Levy Louise E Wilkins-Haug Cecilia Larson Vivian E Shih

OBJECTIVE The objective was to evaluate the relationships between all types of fetal fatty acid oxidation defects and maternal liver disease, including acute fatty liver of pregnancy and hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome. METHODS This was a case-control study comparing fetal fatty acid oxidation defects to the outcome of maternal liver disease. Fifty case i...

Journal: :The Plant cell 1998
A A Millar M Wrischer L Kunst

Transgenic Arabidopsis plants overexpressing the Arabidopsis FATTY ACID ELONGATION1 gene under the control of the 35S promoter from cauliflower mosaic virus accumulated very-long-chain fatty acids (VLCFAs) throughout the plant. In some transformants, C20 and C22 VLCFAs accounted for >30% of the total fatty acids, accumulating at the expense of C16 and C18 fatty acids. These C20 and C22 fatty ac...

Journal: :Cell reports 2015
Chong Yew Tan Samuel Virtue Guillaume Bidault Martin Dale Rachel Hagen Julian L Griffin Antonio Vidal-Puig

Although many transcriptional pathways regulating BAT have been identified, the role of lipid biosynthetic enzymes in thermogenesis has been less investigated. Whereas cold exposure causes changes in the fatty acid composition of BAT, the functional consequences of this remains relatively unexplored. In this study, we demonstrate that the enzyme Elongation of Very Long Chain fatty acids 6 (Elov...

Journal: :Molecular and cellular biology 2003
M C McGuinness J-F Lu H-P Zhang G-X Dong A K Heinzer P A Watkins J Powers K D Smith

Peroxisomal disorders have been associated with malfunction of peroxisomal metabolic pathways, but the pathogenesis of these disorders is largely unknown. X-linked adrenoleukodystrophy (X-ALD) is associated with elevated levels of very-long-chain fatty acids (VLCFA; C(>22:0)) that have been attributed to reduced peroxisomal VLCFA beta-oxidation activity. Previously, our laboratory and others ha...

Journal: :The Plant cell 2005
Huanquan Zheng Owen Rowland Ljerka Kunst

In the absence of cell migration, plant architecture is largely determined by the direction and extent of cell expansion during development. In this report, we show that very-long-chain fatty acid (VLCFA) synthesis plays an essential role in cell expansion. The Arabidopsis thaliana eceriferum10 (cer10) mutants exhibit severe morphological abnormalities and reduced size of aerial organs. These m...

Journal: :Acta medica Iranica 2015
Mehri Najafi Sani Mitra Ahmadi Pejman Roohani Nima Rezaei

Zellweger syndrome (ZS) is a peroxisomal disorder with a multiple congenital anomalies, characterized by stereotypical facies, profound hypotonia, organ involvement including cerebral, retinal, hepatic, and renal. Herein, a 3-month-old female with ZS is presented who was referred because of increased liver enzymes (subclinical hepatitis), which was detected in work-up of her neck cyst, severe h...

2015
Vijitha K. Senanayake Wei Jin Asuka Mochizuki Bassirou Chitou Dayan B. Goodenowe

BACKGROUND Biochemical changes associated with multiple sclerosis (MS), and its various clinical forms have not been characterized well. Therefore, we investigated the biochemistry of MS in relation to its natural history using targeted lipidomics platforms. METHODS Cross-sectional serum samples from 24 secondary progressive (SPMS), 100 relapsing remitting (RRMS), 19 primary progressive MS (P...

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