Primary mitochondrial respiratory chain (RC) diseases are heterogeneous in etiology and manifestations but collectively impair cellular energy metabolism. Mechanism(s) by which RC dysfunction causes global cellular sequelae are poorly understood. To identify a common cellular response to RC disease, integrated gene, pathway, and systems biology analyses were performed in human primary RC disease skeletal muscle and fibroblast transcriptomes. Significant changes were evident in muscle across diverse RC complex and genetic etiologies that were consistent with prior reports in other primary RC disease models and involved dysregulation of genes involved in RNA processing, protein translation, transport, and degradation, and muscle structure. Gl...
<p>(<b>A</b>) <b>Integrated overview modeling general interactions between central nutrient-sensing ...
Hundreds of genes have been associated with respiratory chain disease (RCD), the most common inborn ...
BACKGROUND: Mutations in the gene encoding thymidine kinase 2 (TK2) result in the myopathic form of ...
<div><p>Primary mitochondrial respiratory chain (RC) diseases are heterogeneous in etiology and mani...
<p>(<b>A</b>) The global pattern of differential gene expression showed a highly significant negativ...
Mitochondrial respiratory chain (RC) deficiency is among the most common causes of inherited metabol...
Mitochondria are network-like organelles present in most mammalian cells. They contain the respirato...
Mitochondrial DNA deletions affect energy metabolism at tissue-specific and cell-specific threshold ...
Abstract: Mitochondrial DNA deletions affect energy metabolism at tissue-specific and cell-specific ...
As energy producers, mitochondria play a pivotal role in multiple cellular processes. Although sever...
Respiratory chain complex I (CI) dysfunctions have been recognized as one of the most frequent cause...
Mitochondrial disorders (MDs) are heterogeneous multisystemic disorders due to impaired oxidative ph...
Abstract Reversible infantile respiratory chain deficiency (RIRCD) is a rare mitochondrial myopathy...
AbstractDefects of the mitochondrial protein synthesis cause a subgroup of mitochondrial diseases, w...
<p><b>A</b>) Cytochrome <i>c</i> oxidase (COX) histochemical reactivity revealed a mosaic of COX-def...
<p>(<b>A</b>) <b>Integrated overview modeling general interactions between central nutrient-sensing ...
Hundreds of genes have been associated with respiratory chain disease (RCD), the most common inborn ...
BACKGROUND: Mutations in the gene encoding thymidine kinase 2 (TK2) result in the myopathic form of ...
<div><p>Primary mitochondrial respiratory chain (RC) diseases are heterogeneous in etiology and mani...
<p>(<b>A</b>) The global pattern of differential gene expression showed a highly significant negativ...
Mitochondrial respiratory chain (RC) deficiency is among the most common causes of inherited metabol...
Mitochondria are network-like organelles present in most mammalian cells. They contain the respirato...
Mitochondrial DNA deletions affect energy metabolism at tissue-specific and cell-specific threshold ...
Abstract: Mitochondrial DNA deletions affect energy metabolism at tissue-specific and cell-specific ...
As energy producers, mitochondria play a pivotal role in multiple cellular processes. Although sever...
Respiratory chain complex I (CI) dysfunctions have been recognized as one of the most frequent cause...
Mitochondrial disorders (MDs) are heterogeneous multisystemic disorders due to impaired oxidative ph...
Abstract Reversible infantile respiratory chain deficiency (RIRCD) is a rare mitochondrial myopathy...
AbstractDefects of the mitochondrial protein synthesis cause a subgroup of mitochondrial diseases, w...
<p><b>A</b>) Cytochrome <i>c</i> oxidase (COX) histochemical reactivity revealed a mosaic of COX-def...
<p>(<b>A</b>) <b>Integrated overview modeling general interactions between central nutrient-sensing ...
Hundreds of genes have been associated with respiratory chain disease (RCD), the most common inborn ...
BACKGROUND: Mutations in the gene encoding thymidine kinase 2 (TK2) result in the myopathic form of ...