Amy Sugimoto - Sacramento - ALS Association

Research (continued) & Recipe Box
Recipe Box: Breakfast at its BEST!
Baked French Swirl
Contributed by: Leslee DeFazio
Use 3 Qt. baking dish
1 lb loaf of cinnamon bread (cut into cubes)
3/4 cups dried cranberries
3 cups half and half or milk
6 eggs (beaten)
2 tsp vanilla
Refrigerate 1 hour or overnight
Bake at 350 degrees for 45 minutes
Sprinkle with sugar, cinnamon
Serve with butter and syrup
Makes approximately 7-8 servings
Cheesy Sausage Grits
� 16 c. Water
� 4 c. Grits
� 2 Tbsp. Butter
� 1 tsp. Mexican Oregano, ground
� 1/4 tsp. Chile Powder
� 1 1/2 c. Cheddar Cheese, shredded
� 1 package Jimmy Dean® Country Regular
Roll Sausage, cooked, crumbled, drained
� 3 tsp. Parsley, fresh chopped
� 3 tsp. Cilantro, fresh chopped
Directions: Begin by boiling water in
a small pot with the butter. When
water comes to a boil add grits and
stir continuously. When the grits are
cooked add the butter, Mexican oregano,
chili powder, cheese and sausage.
Garnish with parsley and cilantro.
Makes approximately 7-8 servings
Page 8
Misfolded Mutant SOD1 Directly Inhibits Conductance
in Mouse Model
New research uncovers what may be a primary neuron-damaging
insult that occurs in an inherited form of the devastating neurodegenerative
disorder, amyotrophic lateral sclerosis (ALS). The mutant
superoxide dismutase (SOD1) protein is one cause of familial
ALS. Although mechanisms underlying mutant SOD1 neurotoxicity
remain uncertain, this mutant protein is associated with the
cell’s mitochondria, tiny intracellular energy-producing structures.
Investigators, lead by Professor Don W. Cleveland at the University
of California San Diego, report in the current issue of the
scientific journal Neuron that abnormally folded mutant SOD1
interacts with voltage-dependent anion channel (VDAC1), which
is located in the outer membrane of the mitochondria.
This finding sheds light into possible molecular links between mutant
SOD1 mitochondrial dysfunction and spinal motor neuron
degeneration of inherited ALS. This channel controls important
metabolic and energetic functions between the mitochondria and
the rest of the cell. Interestingly, this same channel has been implicated
in Parkinson’s disease where studies have shown that a
protein linked to the disease called Parkin interacts with this
channel.
“Our evidence demonstrates that reduced VDAC1 function and
correspondingly reduced mitochondrial function are direct components
of intracellular damage from mutant SOD1,” says Professor
Cleveland. “The finding that VDAC1 is a target for mutant
SOD1 within the nervous system provides important insight into
the mechanism underlying premature degeneration and death of
motor neurons.”
These studies were performed in the mutant SOD1 rats using
antibodies that specifically recognize the abnormally folded mutant
form of SOD1. Investigators were able to demonstrate a
reduction in conductance through this channel. The channel conductance
was not affected in liver mitochondria, and previous
studies have shown that abnormally folded mutant protein does
not accumulate in liver mitochondria. This suggests that this interaction
only occurs with the abnormally folded mutant protein
in the affected tissues in ALS. Chronic mitochondrial dysfunction
can lead to oxidative damage and neuronal degeneration.
“This study builds on earlier findings that mitochondria are critical
players in the disease mechanism; however, the new findings highlight
a novel mechanism and a potential new target for the development
of therapies” commented ALS Association Chief Scientist
Lucie Bruijn, Ph.D.