Figure 5.

Rescue of the Biochemical Deficiencies in NFU1 and BOLA3 Mutant Fibroblasts by Retroviral Expression of the Wild-Type cDNAs

(A) Immunoblot of control and mutant NFU1 fibroblast mitochondria transduced with constructs expressing mitochondrial or cytosolic isoforms of NFU1were immunoblotted with antibodies against NFU1, lipoate, complex I subunit NDUFA9, complex II subunit SDHA, and actin.

(B) BN-PAGE analysis of the same samples as in (A).

(C) Immunoblot of control and mutant BOLA3 fibroblast mitochondria transduced with constructs expressing the BOLA3-1 and BOLA3-2 isoforms were immunoblotted with antibodies against NFU1, lipoate, complex I subunit NDUFA9, complex II subunit SDHA, and actin.

(D) BN-PAGE analysis of the same samples as in (C).