Skip to main content

Table 2 Mitochondrial respiratory chain immunofluorescence analysis

From: Axial mitochondrial myopathy in a patient with rapidly progressive adult-onset scoliosis

Complex Antigen Tissue area showing reduced staining1 Partially or totally deficient fibers2
I NDUFS33 >75% Frequent
II SDHB3 25%-75% None
III UQCRC23 <25% Frequent
IV-I MTCO-14 <25% Frequent
IV-IV COX IV3 25%-75% Frequent
V OSCP3 <25% None
PDH PDH E1α3 >75% None
  1. 1“Reduced staining” included totally deficient fibers, partially deficient fibers, and fibers with hypodensity of mitochondria; the involved area was semi-quantified as variable focal (<25% of the tissue area), variable regional (25-75% of the tissue area) and widespread (>75% of the tissue area).
  2. 2For each complex, fibers with strong red (porin) staining but no green staining were considered totally deficient, fibers with patchy green and red staining were considered partially deficient, while fibers lacking both red and green staining were considered to show mitochondrial hypodensity. Frequent was defined as ≥ 10% of muscle fibers in the entire specimen.
  3. 3Nuclear DNA-encoded subunit.
  4. 4mtDNA-encoded subunit.