Gene expression in human healthy control and ISCU myopathy patient muscle biopsies
Ontology highlight
ABSTRACT:
ORGANISM(S): Homo sapiens
SUBMITTER: Chuming Chen
PROVIDER: E-GEOD-48574 | biostudies-arrayexpress |
REPOSITORIES: biostudies-arrayexpress
Publications
Elevated FGF21 secretion, PGC-1α and ketogenic enzyme expression are hallmarks of iron-sulfur cluster depletion in human skeletal muscle.
Crooks Daniel R DR Natarajan Thanemozhi G TG Jeong Suh Young SY Chen Chuming C Park Sun Young SY Huang Hongzhan H Ghosh Manik C MC Tong Wing-Hang WH Haller Ronald G RG Wu Cathy C Rouault Tracey A TA
Human molecular genetics 20130813 1
Iron-sulfur (Fe-S) clusters are ancient enzyme cofactors found in virtually all life forms. We evaluated the physiological effects of chronic Fe-S cluster deficiency in human skeletal muscle, a tissue that relies heavily on Fe-S cluster-mediated aerobic energy metabolism. Despite greatly decreased oxidative capacity, muscle tissue from patients deficient in the Fe-S cluster scaffold protein ISCU showed a predominance of type I oxidative muscle fibers and higher capillary density, enhanced expres ...[more]
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