05-25-2011, 04:13 PM
36. C
Leigh's disease, also known as Subacute Necrotizing Encephalomyelopathy (SNEM), is a rare neurometabolic disorder that affects the central nervous system.
There is another form of this disease called the X-linked Leigh's syndrome (OMIM 308930), which is a mutation in the oxidative phosphorylation enzymes (which are on both the mtDNA and the nuclear DNA). The X-linked Leigh's disease is a mutation of a gene encoding PDHA1, part of the pyruvate dehydrogenase complex, located on the X chromosome
It is an inherited disorder that usually affects infants between the age of three months and two years, but, in rare cases, teenagers and adults as well. In the case of the disease, mutations in mitochondrial DNA (mtDNA) or in nuclear DNA (gene SURF1[2] and some COX assembly factors) cause degradation of motor skills and eventually death.
Mitochondria are an essential organelle in eukaryotic cells. Their function is to convert the potential energy of glucose, amino acids, and fatty acids into adenosine triphosphate (ATP). Mitochondria carry their own DNA, called mitochondrial DNA [mtDNA]. The information stored in the mtDNA is used to produce several of the enzymes essential to the production of ATP.
Mutations in the mtDNA that cause the mitochondria to fail, to function improperly, a person is at risk for a number of disorders, including Leigh's disease. In the case of Leigh's disease, crucial cells in the brain stem have mutated mtDNA, creating poorly functioning mitochondria. This causes a chronic lack of energy in the cells, which, in turn, affects the central nervous system and inhibits motor functions.
http://en.wikipedia.org/wiki/Leigh's_disease
37. B
Mebendazole or MBZ is a benzimidazole drug developed by Janssen Pharmaceutica and marketed as Vermox, Ovex, Antiox, and Pripsen. It is used to treat infestations by worms including pinworms, roundworms, tapeworms, hookworms, and whipworms
Mechanism of action
Mebendazole is thought to work by selectively inhibiting the synthesis of microtubules in parasitic worms, and by destroying extant cytoplasmic microtubes in their intestinal cells: thereby blocking the uptake of glucose and other nutrients, resulting in the gradual immobilization and eventual death of the helminths. The drug is a highly effective broad spectrum antihelminitic indicated for the treatment of nematode infestations, including round worm, whip worm, thread worm, and hook worm. It is poorly absorbed and has no systemic effects.
Leigh's disease, also known as Subacute Necrotizing Encephalomyelopathy (SNEM), is a rare neurometabolic disorder that affects the central nervous system.
There is another form of this disease called the X-linked Leigh's syndrome (OMIM 308930), which is a mutation in the oxidative phosphorylation enzymes (which are on both the mtDNA and the nuclear DNA). The X-linked Leigh's disease is a mutation of a gene encoding PDHA1, part of the pyruvate dehydrogenase complex, located on the X chromosome
It is an inherited disorder that usually affects infants between the age of three months and two years, but, in rare cases, teenagers and adults as well. In the case of the disease, mutations in mitochondrial DNA (mtDNA) or in nuclear DNA (gene SURF1[2] and some COX assembly factors) cause degradation of motor skills and eventually death.
Mitochondria are an essential organelle in eukaryotic cells. Their function is to convert the potential energy of glucose, amino acids, and fatty acids into adenosine triphosphate (ATP). Mitochondria carry their own DNA, called mitochondrial DNA [mtDNA]. The information stored in the mtDNA is used to produce several of the enzymes essential to the production of ATP.
Mutations in the mtDNA that cause the mitochondria to fail, to function improperly, a person is at risk for a number of disorders, including Leigh's disease. In the case of Leigh's disease, crucial cells in the brain stem have mutated mtDNA, creating poorly functioning mitochondria. This causes a chronic lack of energy in the cells, which, in turn, affects the central nervous system and inhibits motor functions.
http://en.wikipedia.org/wiki/Leigh's_disease
37. B
Mebendazole or MBZ is a benzimidazole drug developed by Janssen Pharmaceutica and marketed as Vermox, Ovex, Antiox, and Pripsen. It is used to treat infestations by worms including pinworms, roundworms, tapeworms, hookworms, and whipworms
Mechanism of action
Mebendazole is thought to work by selectively inhibiting the synthesis of microtubules in parasitic worms, and by destroying extant cytoplasmic microtubes in their intestinal cells: thereby blocking the uptake of glucose and other nutrients, resulting in the gradual immobilization and eventual death of the helminths. The drug is a highly effective broad spectrum antihelminitic indicated for the treatment of nematode infestations, including round worm, whip worm, thread worm, and hook worm. It is poorly absorbed and has no systemic effects.