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18 Cards in this Set
- Front
- Back
oxidation of medium chain length fatty acids:
-dietary medium chain length fatty acids are (less/more) water soluble than long chains and (are/are not) stored in adipose as triglyceride -following a meal, they enter liver mitochondrial matrix by ... transporter -activated to acyl CoA derivative and oxidized to acetyl CoA via beta-oxidation |
more
are not monocarboxylate |
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oxidation of FAs with odd-chain lengths:
-beta-oxidation spiral continues until # carbons remain -the 3-carbon ... is produced to consume the last 3 carbons -propionyl CoA converted to TCA intermediate ... (can be used for gluconeogenesis) |
5
propionyl CoA succinyl CoA |
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other types of fatty acid oxidation:
-... beta-oxidation -... alpha-oxidation -... omega-oxidation -these pathways function to decrease levels of water-insoluble fatty acids or toxic xenobiotic (toxic metabolites) fatty acid-like compounds -not feedback regulated --> rate is regulated only by availability of ... |
peroxisomal
peroxisomal microsomal substrate |
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peroxisomes must contain ... to breakdown hydrogen peroxide
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catalase
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Peroxisome functions:
-beta-oxidation of VLCFA (very long chain fatty acid) -biosynthesis of plasmalogen, PAF (platelet activating factor), cholesterol and isoprenoids, DHA -synthesis of ... acids -detoxification of glycolate to ... (excess glycolate leads to precipitation of calcium oxalate --> kidney stones) -peroxisomes are important in preventing kidney stones |
bile
glycine |
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peroxisomal beta-oxidation of fatty acids:
-oxidize VLCFA (24-26 carbons long) into ... more manageable units |
smaller
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beta-oxidation in peroxisomes differs from beta-oxidation in mitochondria in the following ways:
-carnitine is not required -first step is catalyzed by FAD-containing ... -other enzymes have similar actions to their mitochondrial counterparts, but encoded by separate ... -oxidation stops when chain reaches 4-6 carbons, then chain transferred to ... as a carnitine derivative to complete oxidation |
oxidase
genes mitochondria |
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there are 2 categories of peroxisomal disorders:
1) failure to form intact normal peroxisomes 2) deficiencies in a single peroxisomal enzyme -there are 25 known peroxisomal disorders put these into the right category: -Infantile Refsum's disease -Adult Refsum's disease -Zellweger's Syndrome -Neonatal adrenoleukodystrophy |
Failure to form intact normal peroxisomes
-Zellweger's Syndrome -Infantile Refsum's disease -Neonatal adrenoleukodystrophy deficiencies in a single peroxisomal enzyme -adult Refsum's disease |
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which disorder is this?
-aka cerebro-hepato-renal syndrome -congenital absence of peroxisomes -most severe type of peroxisomal disorder (fatal within 1st year of life) -no oxidation of VLCFA -accumulation of C26-C38 polyenolic acids in various tissues |
Zellweger's Syndrome
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symptoms of Zellweger Syndrome:
-... malformations (high forehead, broad nasal bridge,hypoplastic supraorbital ridge, deformed ear lobes) -visual and hearing impairments -hepatomegaly, cirrhosis -mental ... -diminished or absent ... -loss of muscle tone (...) -neonatal ... -renal ... |
craniofacial
retardation reflexes hypotonia seizures cysts |
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which disorder is this?
-most common of the peroxisomal disorders -mutation in ABC transporter involved in uptake of VLCFA across peroxisomal membranes (no beta-oxidation) -results in accumulation of VLCFA in blood and cells |
neonatal and X-linked Adrenoleukodystrophy (ALD)
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symptoms of X-linked ALD:
-1st neurological symptoms occur at #-# years of age -...-like symptoms -visual and auditory disturbances -poor ... -seizures rapid progression to a ... state -impaired adrenal function and deficiency of steroid hormones -damages white matter of brain and impairs the adrenal glands |
4-8
ADHD coordination vegetative |
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treatments of X-linked ALD:
-modification of ... -... oil |
diet
Lorenzo's |
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peroxisomal alpha-oxidation of long-chain branched-chain fatty acids:
-branched chain fatty acids are degradation products of ... -found mostly in ... -... acid and ... acid -peroxisomal alpha-oxidation with alpha-hydroxylase, followed by beta-oxidation spirals |
chlorophyll
green vegetables phytanic pristanic |
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which disorder is this?
-inherited enzyme defect in alpha-oxidation (phytanoyl-CoA hydroxylase) -disease caused by accumulation of phytanic acid in blood and tissue (methyl group on phytanic acid blocks normal beta-oxidation) |
adult Refsum's disease
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symptoms of Refsum's disease:
-usually adult onset, but infantile forms exist -retinitis pigmentosa, progressive ... loss -... (loss of smell) -hearing loss -sensory and peripheral neuropathy -symptoms involve the ... -cardiac abnormalities may be present |
vision
anosmia senses |
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omega-oxidation of fatty acids:
-occurs in the ... microsomes (budded vesicles) -converts fatty acids to ... acids, which can then undergo beta-oxidation in mitochondria |
ER
dicarboxylic |
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which disorder is this?
-deficiency in mitochondrial medium chain acyl-CoA dehydrogenase (MCAD deficiency), impairing beta-oxidation -omega oxidation stimulated, resulting in excess accumulation of dicarboxylic acids in the urine |
dicarboxylic aciduria
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