Table 1.

Source and characteristics of respiration-deficient Chinese hamster fibroblast cell lines

Cell lineDerived fromDefectsReference
CCLI6-B2 Chinese hamster CCLI6 (DON) Mutation in X-linked NDUFA1* gene complex I activity 1%-2% of WT 29  
CCLI6-B9 Chinese hamster CCLI6 (DON) Mutation in small integral membrane anchor protein CII-3 of complex II 30  
  Complex II activity less than 5% of WT  
Gal32 Chinese hamster V79 Mutation in nuclear gene 31  
  Complex IV activity 1%-2% of WT  
  Complex I activity 1%-2% of WT  
  Defective mitochondrial protein synthesis  
V79-G4 Chinese hamster V79 Mutation in nuclear gene on X chromosome 32  
  Complex I activity 1%-2% of WT  
  Different complementation group to CCLI6-B9  
V79-G7 Chinese hamster V79 Mutation in nuclear gene 33  
  Complex IV activity 6.6% of WT  
  Complex V activity 11.6% of WT  
  Defective mitochondrial protein synthesis  
Cell lineDerived fromDefectsReference
CCLI6-B2 Chinese hamster CCLI6 (DON) Mutation in X-linked NDUFA1* gene complex I activity 1%-2% of WT 29  
CCLI6-B9 Chinese hamster CCLI6 (DON) Mutation in small integral membrane anchor protein CII-3 of complex II 30  
  Complex II activity less than 5% of WT  
Gal32 Chinese hamster V79 Mutation in nuclear gene 31  
  Complex IV activity 1%-2% of WT  
  Complex I activity 1%-2% of WT  
  Defective mitochondrial protein synthesis  
V79-G4 Chinese hamster V79 Mutation in nuclear gene on X chromosome 32  
  Complex I activity 1%-2% of WT  
  Different complementation group to CCLI6-B9  
V79-G7 Chinese hamster V79 Mutation in nuclear gene 33  
  Complex IV activity 6.6% of WT  
  Complex V activity 11.6% of WT  
  Defective mitochondrial protein synthesis  
*

Human NDUFA1 on the X chromosome encodes the MWFE polypeptide of mammalian complex I.

Wild type.

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