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Titolo:
CYTOARCHITECTURAL AND METABOLIC ADAPTATIONS IN MUSCLES WITH MITOCHONDRIAL AND CYTOSOLIC CREATINE-KINASE DEFICIENCIES
Autore:
STEEGHS K; OERLEMANS F; DEHAAN A; HEERSCHAP A; VERDOODT L; DEBIE M; RUITENBEEK W; BENDERS A; JOST C; VANDEURSEN J; TULLSON P; TERJUNG R; JAP P; JACOB W; PETTE D; WIERINGA B;
Indirizzi:
UNIV NIJMEGEN,FAC MED SCI,DEPT CELL BIOL & HISTOL,POB 9101 NL-6500 HBNIJMEGEN NETHERLANDS UNIV NIJMEGEN,FAC MED SCI,DEPT CELL BIOL & HISTOL NL-6500 HB NIJMEGENNETHERLANDS UNIV NIJMEGEN,FAC MED SCI,DEPT BIOCHEM NL-6500 HB NIJMEGEN NETHERLANDS UNIV NIJMEGEN,FAC MED SCI,DEPT DIAGNOST RADIOL NL-6500 HB NIJMEGEN NETHERLANDS UNIV NIJMEGEN,FAC MED SCI,DEPT PAEDIAT NL-6500 HB NIJMEGEN NETHERLANDS VRIJE UNIV AMSTERDAM,FAC HUMAN MOVEMENT SCI,INST FUNDAMENTAL & CLIN HUMAN MOVEMENT SCI NL-1081 BT AMSTERDAM NETHERLANDS UNIV INSTELLING ANTWERP,CTR ELECTRONMICROSCOPY B-2610 WILRIJK BELGIUM SUNY SYRACUSE,HLTH SCI CTR,DEPT PHYSIOL SYRACUSE NY 13210 UNIV KONSTANZ,DEPT BIOL D-7834 CONSTANCE GERMANY
Titolo Testata:
Molecular and cellular biochemistry
fascicolo: 1-2, volume: 184, anno: 1998,
pagine: 183 - 194
SICI:
0300-8177(1998)184:1-2<183:CAMAIM>2.0.ZU;2-2
Fonte:
ISI
Lingua:
ENG
Soggetto:
EMBRYONIC STEM-CELLS; CELLULAR-ENERGY METABOLISM; SKELETAL-MUSCLE; SARCOPLASMIC-RETICULUM; MOUSE GENOME; PLASMA-MEMBRANE; CONTACT SITES; CA2+ UPTAKE; HEART-RATE; GENE;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
69
Recensione:
Indirizzi per estratti:
Citazione:
K. Steeghs et al., "CYTOARCHITECTURAL AND METABOLIC ADAPTATIONS IN MUSCLES WITH MITOCHONDRIAL AND CYTOSOLIC CREATINE-KINASE DEFICIENCIES", Molecular and cellular biochemistry, 184(1-2), 1998, pp. 183-194

Abstract

We have blocked creatine kinase (CK) mediated phosphocreatine (PCr) reversible arrow ATP transphosphorylation in mitochondria and cytosol of skeletal muscle by knocking out the genes for the mitochondrial (ScCKmit) and the cytosolic (M-CK) CK isoforms in mice. Animals which carry single or double mutations, if kept and tested under standard laboratory conditions, have surprisingly mild changes in muscle physiology. Strenuous ex vivo conditions were necessary to reveal that MM-CK absence in single and double mutants leads to a partial loss of tetanic force output. Single ScCKmit deficiency has no noticeable effects but in combination the mutations cause slowing of the relaxation rate. Importantly, our studies revealed that there is metabolic and cytoarchitectural adaptation to CK defects in energy metabolism. The effects involvemutation type-dependent alterations in the levels of AMP, IMP, glycogen and phosphomonoesters, changes in activity of metabolic enzymes like AMP-deaminase, alterations in mitochondrial volume and contractile protein (MHC isoform) profiles, and a hyperproliferation of the terminal cysternae of the SR tin tubular aggregates). This suggests that there is a compensatory resiliency of loss-of-function and redirection of flux distributions in the metabolic network for cellular energy in ourmutants.

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Documento generato il 06/04/20 alle ore 02:11:42