Mitochondrial abnormality is seen in -
Ans. is 'd' i.e., Oncocytomao Until now, we have disucssed response of injury to a cell as a unit.o However, Injury to a cell is associated withAlteration in cell organelles orAlteration in cytoskeletonAlteration in cell organelles o Impoant organelles, affected in cell injury are1. Lysosomeso Primary lysosomes are membrane-bound intracellular organelles that contain a variety of hydrolytic enzymes(hydrolases) including acid phosphatase, glucronidase, Sulfatase, ribonuclease and collegenase.o Primary lysosomes fuse with membrane bound vacuoles that contain mateial to be digested, forming secondrylysosome or phagolysosomes.o Lysosomes are involved in the breakdown of phagocytosed material in one of two ways.1. Heterophagy Digestion of materials ingested from outside the cell. It is most common in neutrophils and macrophages.2. Autophagy --> Digestion of cells own components.o In some diseses cells sequester abnormal substances that cannot be completely metabolized by lysosomes --> Lysosomal storage diseases.Example are Pompe disease Fabry disease (option b) Metochromatic leukodystrophy Tay - Sachs disease Gaucher disease Mucopolysaccharidosis Sandhoff disease Niamen pick disease Mucolipoidosis GM2-gangliosidosis Fucosidosis & Mannosidosis q Wolman's disease Krabbe disease (option a)2. Mitochondriao As already explanined, mitochondrial dysfunction plays an impoant role in cell injury and apoptosis. o Abnormalities in mitochondria may also occur in some genetic disorders:? Mitochondria! myopathy 0 Leber's heriditary optic neuropathy Kearns sayre syndrome (Ophthalmoplegia plus) Myoclonic epilepsy with ragged red fibres syndrome MELASo In addition, ceain bening tumors found in salivary gland (oncocytoma), thyroid, parathyroid and kidney consist of cells called oncocytes with abundant enlarged mitochondria, giving cells a distinctly easinophilic appearane.3. Induction (hyperophy) of smooth endoplasmic reticulumo The smooth ER is involved in metabolism of ceain drugs and chemicals.o These agents can cause hyperophy of smooth ER as an adaptive response, eg. prolonged barbiturates use is associated with tolerance due to hyperophy of smooth ER of hepatocytes, which metobolizes the drug. For cystoskeletal alteration see next explanation.
Krabbe's disease
Fabry disease
Fanconi syndrome
Oncocytoma