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18 Cards in this Set

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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
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
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
peroxisomes must contain ... to breakdown hydrogen peroxide
catalase
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
peroxisomal beta-oxidation of fatty acids:
-oxidize VLCFA (24-26 carbons long) into ... more manageable units
smaller
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
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
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
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
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)
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
treatments of X-linked ALD:
-modification of ...
-... oil
diet
Lorenzo's
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
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
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
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
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