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MITOCHONDRION
BY: GWEN KETTENBURG
BIOL 1125
STRUCTURAL BASICS
• Sausage shaped organelles the size of bacteria
• Bounded by two membranes
• Smooth outer membrane
• Inner folded membrane called cristae
• Two compartments with free space
• Matrix – Inside inner membrane
• Ribosomes found here
• Mitochondrial DNA
• Intermembrane space – Lies between the two
mitochondrial membranes
FUNCTIONAL BASICS
• “Powerhouse of the cell”
• Main function is to produce energy in the form of
ATP
• Adenosine Triphosphate
• Formed by metabolizing sugar
• Cellular respiration
• Oxidative metabolism
• Proteins on inner membrane carry
out this process
CELLULAR
RESPIRATION
OTHER FUNCTIONS
• Help maintain proper concentration of calcium
ions within the cellular compartments
• Play an important role in programmed cell death
(apoptosis)
MITOCHONDRIAL DNA
(MDNA)
• Located in the matrix
• Helps the mitochondria divide independently
from the cell
• Contains 37 genes
• mDNA is maternally passed down to a mother’s
offspring
• Mutations in mDNA can lead to many illnesses
such as exercise intolerance
MITOCHONDRIAL
DISEASE
• Does most damage to the cells of the brain,
heart, liver, skeletal muscles, kidney, and the
endocrine and respiratory systems
• Result of inherited or spontaneous mutations in
the mitochondrial DNA
• Symptoms can include loss of motor control,
muscle weakness and pain, poor growth,
diabetes, lactic acidosis, developmental delays,
and susceptibility to infection
OTHER DISEASES LINKED TO
DISFUNCTIONAL MITOCHONDRIA
• Alzheimer’s dementia
• Parkinson’s disease
• Huntington disease
• Diabetes