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A Case of Multiple Mitochondrial Dysfunctions Syndrome 1 and Review of the Literature
American Journal of Medical Genetics Part A, EarlyView.ABSTRACT Multiple mitochondrial dysfunctions syndrome 1 (MMDS1, MIM #605711) due to NFU1 gene defects is an ultra‐rare autosomal recessive inborn error of metabolism associated with reduced function of NFU1 iron–sulfur cluster (ISC) scaffold protein.
Charles R. DiFalco +6 more
wiley +1 more source
Majallah-i Dānishgāh-i ̒Ulūm-i Pizishkī-i Qum, 2019 Background and Objectives: Antioxidant alpha lipoic acid and β-defensin 1 protein may have a positive effect on the quality of sperm parameters. In this study, the effect of sperm incubation with alpha lipoic acid antioxidant and β-defensin 1 protein ...
Reyhaneh Khayamabed, Marzieh Tavalaee,
doaj
Lipoic acid assembly on 2-oxoacid dehydrogenases in E.coli [PDF]
, 2011 Lipoic acid is a covalently attached cofactor essential for the activity of 2-oxoacid dehydrogenases and the glycine cleavage system. In the absence of lipoic acid modification the dehydrogenases are inactive and aerobic metabolism is blocked.
El Haj Hassan, Bachar H.
core
Animals, 2020 Simple Summary In male animals, heat stress causes injury to the testes, resulting in an increase in the number of deformed sperm, a reduction in testosterone production, and consequently, reduced reproductive performance.
Y. Xiong +3 more
semanticscholar +1 more source
Alpha-lipoic acid in liver metabolism and disease.
, 1998 R-alpha-Lipoic acid is found naturally occurring as a prosthetic group in alpha-keto acid dehydrogenase complexes of the mitochondria, and as such plays a fundamental role in metabolism.
L. Packer +5 more
core +1 more source
Effect of alpha lipoic acid on leukotriene A4 hydrolase [PDF]
, 2017 Leukotriene A(4) hydrolase is a soluble enzyme with epoxide hydrolase and aminopeptidase activities catalysing the conversion of leukotriene A(4) to leukotriene B-4 and the hydrolysis of the peptide proline-glycine-proline.
Torres, María José +5 more
core +1 more source
Asian Pacific Journal of ReproductionObjective: To investigate the protective effect of alpha-lipoic acid on lead-induced Sertoli cell injury and its potential molecular mechanisms using primary Sertoli cells from rooster testes. Methods: Primary Sertoli cells from 6-week-old rooster testes
Junrong Li +3 more
doaj +1 more source
Mìžnarodnij Endokrinologìčnij Žurnal, 2012 The article deals with one of most common complications of diabetes mellitus — diabetic polyneuropathy. It is proved that alpha-lipoic acid is a drug of pathogenetic treatment of such pathology.
O.V. Bolshova, O.Ya. Samson
doaj +1 more source
Enzymatic diversity in lipoic acid modification of proteins [PDF]
, 2011 Escherichia coli is one of a few organisms with a well characterized and complete metabolic model for lipoic acid metabolism. In this thesis I make discoveries through analysis of the diversity of lipoic acid metabolic genes in other organisms.
Christensen, Quin H.
core
Encapsulation of α-lipoic acid intochitosan and alginate/gelatin hydrogel microparticles and its in vitro antioxidant activity [PDF]
Hemijska Industrija, 2016 Alpha-lipoic acidis an organosulphur compound well-known for its therapeutic potential and antioxidant properties. However, the effective use of α-lipoic acid depends on biological plasma half-life and its preserving stability, which could be ...
Vidović Bojana B. +6 more
doaj +1 more source