title: VDAC3 has differing mitochondrial functions in two types of striated muscles.
abstract: Voltage-dependent anion channel (VDAC) is an abundant mitochondrial outer membrane protein. In mammals, three VDAC isoforms have been characterized. We have previously reported alterations in the function of mitochondria when assessed in situ in different muscle types in VDAC1 deficient mice (Anflous et al., 2001). In the present report we extend the study to VDAC3 deficient muscles and measure the respiratory enzyme activity in both VDAC1 and VDAC3 deficient muscles. While in the heart the absence of VDAC3 causes a decrease in the apparent affinity of in situ mitochondria for ADP, in the gastrocnemius, a mixed glycolytic/oxidative muscle, the affinity of in situ mitochondria for ADP remains unchanged. The absence of VDAC1 causes multiple defects in respiratory complex activities in both types of muscle. However, in VDAC3 deficient mice the defect is restricted to the heart and only to complex IV. These functional alterations correlate with structural aberrations of mitochondria. These results demonstrate that, unlike VDAC1, there is muscle-type specificity for VDAC3 function and therefore in vivo these two isoforms may fulfill different physiologic functions.
authors.affiliation: Department of Molecular and Human Genetics, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA. kanflous@bcm.tmc.edu
authors.forename: Keltoum
authors.initials: K
authors.lastname: Anflous-Pharayra
authors.forename: Nha
authors.initials: N
authors.lastname: Lee
authors.forename: Dawna L
authors.initials: DL
authors.lastname: Armstrong
authors.forename: William J
authors.initials: WJ
authors.lastname: Craigen
mesh_terms: D000244:Adenosine Diphosphate / Q000378:metabolism; D000818:Animals; D001425:Bacterial Outer Membrane Proteins / Q000235:genetics / Q000378:metabolism; D003576:Electron Transport Complex IV / Q000235:genetics / Q000378:metabolism; D051379:Mice; D018345:Mice, Knockout; D008854:Microscopy, Electron; D008928:Mitochondria / Q000201:enzymology*; D033681:Mitochondrial Membrane Transport Proteins; D024101:Mitochondrial Proteins / Q000172:deficiency* / Q000235:genetics* / Q000378:metabolism; D054792:Muscle, Striated / Q000201:enzymology / Q000648:ultrastructure; D009206:Myocardium / Q000201:enzymology / Q000648:ultrastructure; D010101:Oxygen Consumption; D050994:Voltage-Dependent Anion Channels / Q000172:deficiency* / Q000235:genetics* / Q000378:metabolism
chemical_list: D001425:Bacterial Outer Membrane Proteins; D033681:Mitochondrial Membrane Transport Proteins; D024101:Mitochondrial Proteins; C480078:Vdac3 protein, mouse; D050994:Voltage-Dependent Anion Channels; D000244:Adenosine Diphosphate; D003576:Electron Transport Complex IV