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Synergistic heterozygosity in mice with inherited enzyme deficiencies of mitochondrial fatty acid β-oxidation

We have used mice with inborn errors of mitochondrial fatty acid ?-oxidation to test the concept of synergistic heterozygosity. We postulated that clinical disease can result from heterozygous mutations in more than one gene in single or related metabolic pathways.

出版机构:Elsevier Science

刊物名称:Molecular Genetics and Metabolism

出版时间:2005

ISSN:1096-7192

数据源:报刊文摘

The structure and function of oxidized albumin in hemodialysis patients: Its role in elevated oxidative stress via neutrophil burst

Oxidized albumin is a reliable marker of oxidative stress in hemodialysis (HD) patients. However, oxidized albumin in vivo and its possible clinical significance has been rarely investigated.

出版机构:Elsevier Science

刊物名称:Biochemical and Biophysical Research Communications

出版时间:2005

ISSN:0006-291X

数据源:报刊文摘

Differential inhibitory effect of long-chain acyl-CoA esters on succinate and glutamate transport into rat liver mitochondria and its possible implications for long-chain fatty acid oxidation defects

Long-chain fatty acid �-oxidation defects are associated with a series of clinical and biochemical abnormalities, including accumulation of long-chain acyl-CoA esters which have been shown to inhibit several enzymes and transport systems that may disturb energy metabolism.

出版机构:Elsevier Science

刊物名称:Molecular Genetics and Metabolism

出版时间:2005

ISSN:1096-7192

数据源:报刊文摘

Down-regulation of Hsp60 expression by RNAi impairs folding of medium-chain acyl-CoA dehydrogenase wild-type and disease-associated proteins

We have analyzed the role of the highly abundant molecular chaperone Hsp60 in the biogenesis of medium-chain acyl-CoA dehydrogenase (MCAD) using RNA interference (RNAi).

出版机构:Elsevier Science

刊物名称:Molecular Genetics and Metabolism

出版时间:2005

ISSN:1096-7192

数据源:报刊文摘

Accumulated BH4 in mouse liver caused by administration of either 6R- or 6SBH4 consisted solely of the 6R-diastereomer: Evidence of oxidation to BH2 and enzymic reduction

Mice were given (i.p.) L-erythro-(6S)-tetrahydrobiopterin (6SBH4) or 6RBH4 and the increase in liver BH4 in both groups was almost the same. The C6-chirality of liver BH4 was determined by HPLC. After administration of 6SBH4, the liver BH4 consisted mainly of 6RBH4 (>95%).

出版机构:Elsevier Science

刊物名称:Molecular Genetics and Metabolism

出版时间:2005

ISSN:1096-7192

数据源:报刊文摘

dif-1 and colt, both implicated in early embryonic development, encode carnitine acylcarnitine translocase

It has always been assumed that during development the embryo and fetus depend only on glycolysis for energy generation and that they do not oxidize fatty acids. Recently, however, we found abundant expression and activity of fatty acid oxidation (FAO) enzymes in the human embryo and fetus.

出版机构:Elsevier Science

刊物名称:Molecular Genetics and Metabolism

出版时间:2005

ISSN:1096-7192

数据源:报刊文摘

Differential induction of genes in liver and brown adipose tissue regulated by peroxisome proliferator-activated receptor-α during fasting and cold exposure in acyl-CoA dehydrogenase-deficient mice

Mice deficient for either long-chain acyl-CoA dehydrogenase (LCAD?/?) or very-long-chain acyl-CoA dehydrogenase (VLCAD?/?) develop hepatic steatosis upon fasting, due to disrupted mitochondrial fatty acid oxidation.

出版机构:Elsevier Science

刊物名称:Molecular Genetics and Metabolism

出版时间:2005

ISSN:1096-7192

数据源:报刊文摘

Paraoxonase-1 and linoleic acid oxidation in familial hypercholesterolemia

Serum paraoxonase-1 (PON1) is a high-density lipoprotein-associated enzyme that can inhibit low-density lipoprotein (LDL) oxidation in vitro. The role of PON1 in vivo still remains to be clarified. We investigated the effect of PON1 genotype (?

出版机构:Elsevier Science

刊物名称:Biochemical and Biophysical Research Communications

出版时间:2005

ISSN:0006-291X

数据源:报刊文摘

α-Lipoic acid increases insulin sensitivity by activating AMPK in skeletal muscle

Triglyceride accumulation in skeletal muscle contributes to insulin resistance in obesity. We recently showed that ?-lipoic acid (ALA) reduces body weight and prevents the development of diabetes in diabetes-prone obese rats by reducing triglyceride accumulation in non-adipose tissues.

出版机构:Elsevier Science

刊物名称:Biochemical and Biophysical Research Communications

出版时间:2005

ISSN:0006-291X

数据源:报刊文摘

Structure of the cytochrome complex SoxXA of Paracoccus pantotrophus, a heme enzyme initiating chemotrophic sulfur oxidation

The sulfur-oxidizing enzyme system (Sox) of the chemotroph Paracoccus pantotrophus is composed of several proteins, which together oxidize hydrogen sulfide, sulfur, thiosulfate or sulfite and transfers the gained electrons to the respiratory chain.

出版机构:Elsevier Science

刊物名称:Journal of Structural Biology

出版时间:2005

ISSN:1047-8477

数据源:报刊文摘