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

Table 2 Mitochondrial respiratory chain immunofluorescence analysis

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“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).
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.
³Nuclear DNA-encoded subunit.
⁴mtDNA-encoded subunit.

ISSN: 2051-5960