Use of gene targeting for compromising energy homeostasis in neuro-muscular tissues: The role of sarcomeric mitochondrial creatine kinase

Karen Steeghs; Arend Heerschap; Arnold De Haan; Wim Ruitenbeek; Frank Oerlemans; Jan Van Deursen; Benjamin Perryman; Dirk Pette; Marloes Brückwilder; Jolande Koudijs; Jap Paul; Bé Wieringa

doi:10.1016/S0165-0270(96)00124-0

Use of gene targeting for compromising energy homeostasis in neuro-muscular tissues: The role of sarcomeric mitochondrial creatine kinase

Karen Steeghs, Arend Heerschap, Arnold De Haan, Wim Ruitenbeek, Frank Oerlemans, Jan Van Deursen, Benjamin Perryman, Dirk Pette, Marloes Brückwilder, Jolande Koudijs, Jap Paul, Bé Wieringa

Pediatric and Adolescent Medicine

Research output: Contribution to journal › Article › peer-review

42 Scopus citations

Abstract

We have introduced a single knock-out mutation in the mitochondrial creatine kinase gene (ScCKmit) in the mouse germ line via targeted mutagenesis in mouse embryonic stem (ES) cells. Surprisingly, ScCKmit -/- muscles, unlike muscles of mice with a deficiency of cytosolic M-type creatine kinase (M-CK -/-; Van Deursen et al. (1993) Cell 74, 621-631), display no altered morphology, performance or oxidative phosphorylation capacity. Also, the levels of high energy phosphate metabolites were essentially unaltered in ScCKmit mutants. Our results challenge some of the present concepts about the strict coupling between CKmit function and aerobic respiration.

Original language	English (US)
Pages (from-to)	29-41
Number of pages	13
Journal	Journal of Neuroscience Methods
Volume	71
Issue number	1
DOIs	https://doi.org/10.1016/S0165-0270(96)00124-0
State	Published - Jan 1997

Keywords

creatine kinase
energy homeostasis
gene targeting
mitochondria
muscle physiology
oxidative phosphorylation

ASJC Scopus subject areas

General Neuroscience

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10.1016/S0165-0270(96)00124-0

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Steeghs, K., Heerschap, A., De Haan, A., Ruitenbeek, W., Oerlemans, F., Van Deursen, J., Perryman, B., Pette, D., Brückwilder, M., Koudijs, J., Paul, J., & Wieringa, B. (1997). Use of gene targeting for compromising energy homeostasis in neuro-muscular tissues: The role of sarcomeric mitochondrial creatine kinase. Journal of Neuroscience Methods, 71(1), 29-41. https://doi.org/10.1016/S0165-0270(96)00124-0

Steeghs, K, Heerschap, A, De Haan, A, Ruitenbeek, W, Oerlemans, F, Van Deursen, J, Perryman, B, Pette, D, Brückwilder, M, Koudijs, J, Paul, J & Wieringa, B 1997, 'Use of gene targeting for compromising energy homeostasis in neuro-muscular tissues: The role of sarcomeric mitochondrial creatine kinase', Journal of Neuroscience Methods, vol. 71, no. 1, pp. 29-41. https://doi.org/10.1016/S0165-0270(96)00124-0

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abstract = "We have introduced a single knock-out mutation in the mitochondrial creatine kinase gene (ScCKmit) in the mouse germ line via targeted mutagenesis in mouse embryonic stem (ES) cells. Surprisingly, ScCKmit -/- muscles, unlike muscles of mice with a deficiency of cytosolic M-type creatine kinase (M-CK -/-; Van Deursen et al. (1993) Cell 74, 621-631), display no altered morphology, performance or oxidative phosphorylation capacity. Also, the levels of high energy phosphate metabolites were essentially unaltered in ScCKmit mutants. Our results challenge some of the present concepts about the strict coupling between CKmit function and aerobic respiration.",

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AU - Ruitenbeek, Wim

AU - Oerlemans, Frank

AU - Van Deursen, Jan

AU - Perryman, Benjamin

AU - Pette, Dirk

AU - Brückwilder, Marloes

AU - Koudijs, Jolande

AU - Paul, Jap

AU - Wieringa, Bé

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AB - We have introduced a single knock-out mutation in the mitochondrial creatine kinase gene (ScCKmit) in the mouse germ line via targeted mutagenesis in mouse embryonic stem (ES) cells. Surprisingly, ScCKmit -/- muscles, unlike muscles of mice with a deficiency of cytosolic M-type creatine kinase (M-CK -/-; Van Deursen et al. (1993) Cell 74, 621-631), display no altered morphology, performance or oxidative phosphorylation capacity. Also, the levels of high energy phosphate metabolites were essentially unaltered in ScCKmit mutants. Our results challenge some of the present concepts about the strict coupling between CKmit function and aerobic respiration.

KW - creatine kinase

KW - energy homeostasis

KW - gene targeting

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KW - muscle physiology

KW - oxidative phosphorylation

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Use of gene targeting for compromising energy homeostasis in neuro-muscular tissues: The role of sarcomeric mitochondrial creatine kinase

Abstract

Keywords

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