Notes in Lipid Mobilization and Oxidation

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Published	08/15/2023	Fatty acid oxidation primarily takes place in the {{c1::mitochondria}} of the {{c2::heart, liver, and skeletal muscle}}.
Published	08/15/2023	{{c1::Small and medium fatty acids}} can be transported into the mitochondrial matrix without the carnitine shuttle.
Published	08/15/2023	The {{c1::carnitine shuttle}} transports {{c2::large fatty acids}} across the mitochondrial membranes into the matrix of target cells.
Published	08/15/2023	Each cycle of beta oxidation creates {{c1::1 FADH2, 1 NADH, and 1 acetyl-CoA molecule}}
Published	08/15/2023	Explain the last step of beta oxidation for an odd numbered carbon fatty acid.
Published	08/15/2023	The rate limiting step of fatty acid oxidation is {{c1::the conversion of fatty acid CoA into long chain acylcarnitine}}
Published	08/15/2023	The rate limiting enzyme of {{c1::fatty acid oxidation}} is {{c2::CAT1}}
Published	08/15/2023	Oxidation of {{c1::very long}} fatty acids occurs in {{c2::peroxisomes}}.
Published	08/15/2023	The {{c1::citrate shuttle}} transports {{c2::acetyl CoA and oxaloacetate}} from the matrix into the cytoplasm.
Published	08/15/2023	The two most common{{c1:: fatty acid metabolism disorders }}are {{c2::systemic carnitine deficiency and medium chain acyl-CoA dehydrogenase deficiency…
Published	08/15/2023	{{c1::MCADD}} is caused by a mutation in the {{c2::ACADM gene}} that codes for the {{c3::medium chain acyl-CoA dehydrogenase}}
Published	08/15/2023	The inheritance pattern of MCADD is {{c1::autosomal recessive}}.
Published	08/15/2023	{{c1::MCADD}} is typically diagnosed during {{c2::newborn infant blood tests}} with high levels of {{c3::fatty-acyl carnitine}}.
Published	08/15/2023	MCADD treatment typically includes avoiding {{c1::fasting and prolonged exercise}} and keeping a {{c2::low fat, high carbohydrate }}diet.
Published	08/15/2023	{{c1::Liver cells}} have more {{c2::peroxisomes}} than other cells in the body.
Published	08/15/2023	{{c1::Alpha}}-oxidation occurs exclusively in the peroxisomes and {{c2::beta}}-oxidation occurs in the peroxisomes and mitochondria.
Published	08/15/2023	Zellweger syndrome has an inheritance pattern of {{c1::autosomal recessive}}
Published	08/15/2023	{{c1::Zellweger sydrome}} is caused by a mutation in the {{c2::PEX genes}} that code for peroxins
Published	08/15/2023	{{c1::Peroxins}} are crucial to the ability to form {{c2::peroxisomes.}}
Published	08/15/2023	Patients with {{c1::Zellweger syndrome }}have a buildup of {{c2::peroxins}} in their tissues and organs.
Published	08/15/2023	Zellweger syndrome positive patients often experience {{c1::hepatomegaly }}and corresponding {{c2::jaundice}}.
Published	08/15/2023	{{c1::Refsum disease}} is caused by a mutation in the {{c2::PHYH gene }}that codes for {{c3::phytanoyl-CoA hydroxylase}}, crucial in alpha oxidation.
Published	08/15/2023	The inheritance pattern of Refsum disease is {{c1::autosomal recessive}}.
Published	08/15/2023	Common neurological sympoms associated with {{c1::Refsum disease}} include{{c2:: ataxia and peripheral neuropathy}}.
Published	08/15/2023	Accumulation of {{c1::phytanic acid }}in the {{c2::retina}} associated with {{c3::Refsum disease }}leads to{{c4:: cataracts and retinitis pigmentosa (…
Published	08/15/2023	Skin manifestations of {{c1::Refsum disease}} include {{c2::ichthyosis}}
Published	08/15/2023	Manifestions of {{c1::Refsum disease}} at the bones include {{c2::epiphyseal dysplasia}} and {{c3::shortening of the fourth toe}}
Published	08/15/2023	Diagnosis of {{c1::Refsum disease}} is found through {{c2::high phytanic acid levels in the blood }}or {{c3::lipid vacuoles in epidermal cells from a …
Published	08/15/2023	{{c1::Adrenoleukodystrophy}} is caused by a mutation of the {{c2::ABCD1 gene }}on the {{c3::X}} chromosome.
Published	08/15/2023	The inheritance pattern of adrenoleukodystrophy is {{c1::X linked recessive}}
Published	08/15/2023	{{c1::ABCD1 gene}} affected in {{c2::adrenoleukodystrophy}} codes for the {{c3::ALD transporter}} that transports very long fatty acid chains into per…
Published	08/15/2023	{{c1::Adrenoleukodystrophy}} primarily affects cells of the {{c2::adrenal cortex, the Leydig cells of the testes, and myelin producing cells in white …
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