Mitochondrial Disease

Question 1

Mitochondria

Answer 1

derived from mother
each has dsDNA w/ 13 proteins
mutations occur, spread slowly since they divide like bacteria
some cells may carry mostly mutant mitochondria

Question 2

Mitochondrial Encephalopathies

Answer 2

genetic disease of mitochondria affect tissues with high metabolic rates -> brain + muscle

variable patterns of inheritance = all children inherit from the mother

delayed + progressive course

neurologic signs

bilateral symmetry, white matter necrosis

Question 3

Mytochondrial Myopathies

Answer 3

very few reports -> in dogs + horses

‘ragged red fibers’ in muscle tissues -> represent clusters of abnormal mitochondria

‘parking lot’ paracrystalline arrays in mitochondria -> no cristae = uncertain significance

excercise intolerance

Question 4

Oncocytoma

Answer 4

tumor cells filled with compact masses of mitochondria

Question 5

Autophagy

Answer 5