Rasmussen 1996 Biochem J

From Bioblast
Jump to: navigation, search
Publications in the MiPMap
Rasmussen UF, Rasmussen HN, Andersen AJ, Fogd Jørgensen P, Quistorff B. (1996) Characterization of mitochondria from pig muscle: higher activity of exo-NADH oxidase in animals suffering from malignant hyperthermia. Biochem J 315:659-63.

» PMID: 8615844

Rasmussen UF, Rasmussen HN, Andersen AJ, Fogd Joergensen P, Quistorff B. (1996) Biochem J

Abstract: Mitochondria were isolated from biopsies of the biceps femoris muscle of Danish landrace pigs. Three groups of animals were compared: (1) normal pigs; (2) pigs that were homozygous with respect to the gene Hal(n)/Hal(n) coding for the porcine malignant hyperthermia syndrome; and (3) heterozygote animals. A newly developed micro-method for preparation and assaying of small quantities of intact mitochondria was employed. With this technique mitochondria from biopsies weighing less than 100 mg were examined with respect to cytochrome content as well as phosphorylating and respiratory activities, including the nonphosphorylating exo-NADH oxidase activity. The mitochondria, prepared in a yield of 48%, showed high respiratory activities with tricarboxylic acid-cycle intermediates and pyruvate, and somewhat lower activity with palmitoyl-carnitine as substrate. The ATP synthase activity was about 1000 micromol ATP/min per g of protein and the maximal respiratory activity approx. 700 micromol of O2/min per g of protein. No differences among the three groups of animals were detected, except for the exo-NADH oxidase activities, which were 43, 78 and 107 micromol of O2/min per g of protein in the groups of normal, heterozygous and homozygous animals respectively. It is concluded that the exo-NADH oxidase activity may be a genetic manifestation of malignant hyperthermia and may play a significant role in the heat production characteristic of the syndrome.

Cited by

Gnaiger 2020 BEC MitoPathways


Gnaiger E (2020) Mitochondrial pathways and respiratory control. An introduction to OXPHOS analysis. 5th ed. Bioenerg Commun 2020.2:112 pp. doi:10.26124/bec:2020-0002


Labels: MiParea: Respiration  Pathology: Inherited, Other 

Organism: Pig  Tissue;cell: Skeletal muscle  Preparation: Isolated mitochondria 

Regulation: Substrate  Coupling state: LEAK, OXPHOS  Pathway: N, S, CIV, NS 


BEC 2020.2