Mitochondrial structure and function in the untreated Jackson toxic milk (tx-j) mouse, a model for Wilson disease
EA Roberts, BH Robinson, S Yang - Molecular genetics and metabolism, 2008 - Elsevier
Structural changes in hepatocellular mitochondria are characteristic of Wilson disease (WD).
Features include variability in size and shape, increased density of matrix, discreet
inclusions, and cystic dilatation of the cristae. We examined the functional basis for these
mitochondrial changes in the toxic milk (tx-j) mouse model for WD. Its normal syngeic strain,
C3H, served as control. Hepatic histology was near-normal in tx-j mice at 3–4-months-old
and showed mild inflammation and steatosis at 6-months-old. Transmission electron …
Features include variability in size and shape, increased density of matrix, discreet
inclusions, and cystic dilatation of the cristae. We examined the functional basis for these
mitochondrial changes in the toxic milk (tx-j) mouse model for WD. Its normal syngeic strain,
C3H, served as control. Hepatic histology was near-normal in tx-j mice at 3–4-months-old
and showed mild inflammation and steatosis at 6-months-old. Transmission electron …