nemoci-sympt/METABOLISMUS/mitochondrie/zhorseni-mitochondrialni-funkce
Mitochondrie a stres
- Stresy na mitochondrie:
- Proteotoxic
- Energetic
- Osmotic
- Oxidative [8]
- Existence of multiple quality control mechanisms and adaptive pathways
- mitochondrial biogenesis
- mitochondrial dynamics (fission/fusion)
- Mitophagy
- mitochondrial unfolded protein response (UPRmt) [8]
- Chronic overload of these quality control pathways
- Major cause for mitochondrial dysfunction
- Contributes to the pathogenesis of mitochondrial diseases
- Rare inherited mitochondrial diseases
- Palette of common age-related disorders
- Metabolic diseases
- Neurodegeneration
- Cancer etc.
Mitochondrie a stres
- Stresy na mitochondrie:
- Proteotoxic
- Energetic
- Osmotic
- Oxidative [8]
- Existence of multiple quality control mechanisms and adaptive pathways
- mitochondrial biogenesis
- mitochondrial dynamics (fission/fusion)
- Mitophagy
- mitochondrial unfolded protein response (UPRmt) [8]
- Chronic overload of these quality control pathways
- Major cause for mitochondrial dysfunction
- Contributes to the pathogenesis of mitochondrial diseases
- Rare inherited mitochondrial diseases
- Palette of common age-related disorders
- Metabolic diseases
- Neurodegeneration
- Cancer etc.
Alcohol
- Hasten the progression of some mitochondrial disorders
Alcohol
- Hasten the progression of some mitochondrial disorders
Antibiotika toxická vůči mitochondriím
Antibiotika toxická vůči mitochondriím
Fe
Omezit železo a jeho vstřebávání z červeného masa
Fe
Omezit železo a jeho vstřebávání z červeného masa
MSG (monosodium glutamate)
- Cause migraine headaches in otherwise healthy individuals
- May trigger these events in susceptible people with mitochondrial disease [5]
- Frequently added to Chinese (and other Asian) foods
- In high levels of dried and canned soup [5]
- Avoid it
MSG (monosodium glutamate)
- Cause migraine headaches in otherwise healthy individuals
- May trigger these events in susceptible people with mitochondrial disease [5]
- Frequently added to Chinese (and other Asian) foods
- In high levels of dried and canned soup [5]
- Avoid it
Hydrogen sulfide - H2S
- Forms a complex bond to the ferric moiety
- Causing inhibition of mitochondrial cytochrome oxidase (iron-containing protein)
- Arresting aerobic metabolism in an effect similar to cyanide toxicity
- Very high lipid solubility allows it to penetrate easily through biologic membranes.
- As a cellular poison affects all organs
- Particularly the CNS and pulmonary system
- Spectrum of illness depends on the concentration and duration of exposure
- High concentrations (>700 ppm or >975 mg/m3)
- Potential to cause sudden death
- Effect on the brainstem respiratory center [10]
Zdroj
- Střevní bakterie
- Taňový metabolismus sirných AMK
- Byproduct of the petroleum, viscose rayon, rubber, and mining industries
- Petroleum industry is responsible for most cases of hydrogen sulfide toxicity in North America
- Organic decomposition of sulfur compounds in sewers, barns, liquid manure pits, ships' holds, and sulfur springs
- In caves, sulfur springs, underground deposits of natural gas, or as result of volcanic eruptions.
- Committing suicide became a trend in Japan in 2007, spread to the United States
- Bath sulfur was mixed with toilet bowl cleaner to produce the gas
- Acidic detergent as a proton donor
- Sulfur source such as a pesticide or bath salts
- Rescue workers and others entering the space have been affected [10]
Symptomy
- Low-level exposures to hydrogen sulfide gas
- Local eye and mucous membrane irritation [10]
- Cough
- Dyspnea
- Vertigo
- Confusion
- Nausea and vomiting
- Possible loss of consciousness
- Hemoptysis [10]
- High-level exposures
- Rapidly produce fatal systemic toxicity
- 700-800 ppm or greater
- Loss of consciousness
- Cardiopulmonary arrest [10]
- Complications:
- Acute respiratory distress syndrome
- Acute myocardial infarction
- Sudden loss of consciousness ("knockdown")
- Seizure
- Cardiopulmonary arrest [10]
- Delayed neuropsychiatric sequelae [10]
- Long-term neurologic sequelae from hydrogen sulfide exposure
- Unknown but appears to be linked to longer sublethal exposures
- Paradoxically, high-concentration exposures of hydrogen sulfide may have no long-term effects [10]
Hydrogen sulfide - H2S
- Forms a complex bond to the ferric moiety
- Causing inhibition of mitochondrial cytochrome oxidase (iron-containing protein)
- Arresting aerobic metabolism in an effect similar to cyanide toxicity
- Very high lipid solubility allows it to penetrate easily through biologic membranes.
- As a cellular poison affects all organs
- Particularly the CNS and pulmonary system
- Spectrum of illness depends on the concentration and duration of exposure
- High concentrations (>700 ppm or >975 mg/m3)
- Potential to cause sudden death
- Effect on the brainstem respiratory center [10]
Zdroj
- Střevní bakterie
- Taňový metabolismus sirných AMK
- Byproduct of the petroleum, viscose rayon, rubber, and mining industries
- Petroleum industry is responsible for most cases of hydrogen sulfide toxicity in North America
- Organic decomposition of sulfur compounds in sewers, barns, liquid manure pits, ships' holds, and sulfur springs
- In caves, sulfur springs, underground deposits of natural gas, or as result of volcanic eruptions.
- Committing suicide became a trend in Japan in 2007, spread to the United States
- Bath sulfur was mixed with toilet bowl cleaner to produce the gas
- Acidic detergent as a proton donor
- Sulfur source such as a pesticide or bath salts
- Rescue workers and others entering the space have been affected [10]
Symptomy
- Low-level exposures to hydrogen sulfide gas
- Local eye and mucous membrane irritation [10]
- Cough
- Dyspnea
- Vertigo
- Confusion
- Nausea and vomiting
- Possible loss of consciousness
- Hemoptysis [10]
- High-level exposures
- Rapidly produce fatal systemic toxicity
- 700-800 ppm or greater
- Loss of consciousness
- Cardiopulmonary arrest [10]
- Complications:
- Acute respiratory distress syndrome
- Acute myocardial infarction
- Sudden loss of consciousness ("knockdown")
- Seizure
- Cardiopulmonary arrest [10]
- Delayed neuropsychiatric sequelae [10]
- Long-term neurologic sequelae from hydrogen sulfide exposure
- Unknown but appears to be linked to longer sublethal exposures
- Paradoxically, high-concentration exposures of hydrogen sulfide may have no long-term effects [10]
Hladovění
- Perhaps the most important part of the treatment - vyhnout se hladovění
- Even an overnight “fast” from 8 pm to 8 am may be dangerous in some patients
- If vomiting or loss of appetite (like the flu)
- Should be hospitalized to ensure continuous nutrition (intravenous glucose, for example).
- Small frequent meals may be better than 3-meal-a-day routine for some patients.
- A snack before bedtime may be helpful for some patients
- Complex carbohydrate
- Cornstarch
- Low-sugar rice pudding thickened with a lot of cornstarch
- Pasta, a peanut butter sandwich, bread and butter, unsweetened cereal (oatmeal) or a sandwich are acceptable
- Many patients benefit by being woken up in the middle of the night for a small meal.
Hladovění
- Perhaps the most important part of the treatment - vyhnout se hladovění
- Even an overnight “fast” from 8 pm to 8 am may be dangerous in some patients
- If vomiting or loss of appetite (like the flu)
- Should be hospitalized to ensure continuous nutrition (intravenous glucose, for example).
- Small frequent meals may be better than 3-meal-a-day routine for some patients.
- A snack before bedtime may be helpful for some patients
- Complex carbohydrate
- Cornstarch
- Low-sugar rice pudding thickened with a lot of cornstarch
- Pasta, a peanut butter sandwich, bread and butter, unsweetened cereal (oatmeal) or a sandwich are acceptable
- Many patients benefit by being woken up in the middle of the night for a small meal.
Cigarette smoke
- Carbon monoxide
- Known to hasten the progression of some conditions
- Kills by inhibiting complex IV of OXPHOS [5]
Cigarette smoke
- Carbon monoxide
- Known to hasten the progression of some conditions
- Kills by inhibiting complex IV of OXPHOS [5]
Nedostatek spánku
- Lack of sleep may possibly be harmful [5]
Nedostatek spánku
- Lack of sleep may possibly be harmful [5]
Nežádoucí účinky na mtch
- Deleterious action on mitochondria
- Hyperlactemia
- Metabolic acidosis
- Peripheral neuropathy
- Inhibition of mammalian mitochondrial protein synthesis
- Similar in mitochondria from all tissues studied [9]
Oxidační stres
- Vedlejší produkt buněčného dýchání - kyslíkové radikály
- Mohou způsobit četné mutace v mitochondriální DNA
- Defektnější molekuly vznikají
- Degenerativní změny prohlubovat
- Pozorováno během lidského stárnutí
- Některé změny mohou být dávány do souvislosti s
- Parkinsonovou chorobou [3]
- Vznikem nádorů
Oxidační stres
- Vedlejší produkt buněčného dýchání - kyslíkové radikály
- Mohou způsobit četné mutace v mitochondriální DNA
- Defektnější molekuly vznikají
- Degenerativní změny prohlubovat
- Pozorováno během lidského stárnutí
- Některé změny mohou být dávány do souvislosti s
- Parkinsonovou chorobou [3]
- Vznikem nádorů
All physiologic stressful conditions
- One should not attempt to do so
Cold Stress
- Extremely important
- Thermal regulation (temperature control) is not always normal in people with mitochondrial diseases
- Exposure to cold can result in severe heat loss and trigger an energy crisis
- All exposed body parts should be covered
- Exposure to extreme cold should be avoided [5]
Heat Stress
- Can be a problem in some people
- Those with an inability to sweat normally
- Heat exhaustion and heat stroke may occur on hot days
- Light clothing is important
- Avoid direct sunlight on hot days
- An air-conditioned environment may be needed [5]
All physiologic stressful conditions
- One should not attempt to do so
Cold Stress
- Extremely important
- Thermal regulation (temperature control) is not always normal in people with mitochondrial diseases
- Exposure to cold can result in severe heat loss and trigger an energy crisis
- All exposed body parts should be covered
- Exposure to extreme cold should be avoided [5]
Heat Stress
- Can be a problem in some people
- Those with an inability to sweat normally
- Heat exhaustion and heat stroke may occur on hot days
- Light clothing is important
- Avoid direct sunlight on hot days
- An air-conditioned environment may be needed [5]