Carnitine deficiency can occur particularly in the newborn— and especially in preterm infants—owing to inadequate biosynthesis or renal leakage. Losses can also occur in hemodialysis. This suggests there may be a vitamin-like dietary requirement for carnitine in some individuals. Symptoms of deficiency include hypoglycemia, which is a consequence of impaired fatty acid oxidation, and lipid accumulation with muscular weakness. Treatment is by oral supplementation with carnitine.

Inherited CPT-I deficiency affects only the liver, resulting in reduced fatty acid oxidation and ketogenesis, with hypo glycemia. CPT-II deficiency affects primarily skeletal muscle and, when severe, the liver. The sulfonylurea rugs (glyburide [glibenclamide] and tolbutamide), used in the treatment of Type 2 diabetes mellitus, reduce fatty acid oxidation and, therefore, hyperglycemia by inhibiting CPT-I.

Inherited defects in the enzymes of β-oxidation and keto genesis also lead to nonketotic hypoglycemia, coma, and fatty liver. Defects have been identified in long- and short-chain 3-hydroxyacyl-CoA dehydrogenase (deficiency of the long chain enzyme may be a cause of acute fatty liver of pregnancy). 3-Ketoacyl-CoA thiolase and HMG-CoA lyase deficiency also affect the degradation of leucine, a ketogenic amino acid .

Jamaican vomiting sicknessis caused by eating the unripe fruit of the akee tree, which contains the toxin hypoglycin. This inactivates medium- and short-chain acyl-CoA dehydrogenase, inhibiting β-oxidation and causing hypoglycemia. Dicarboxylic aciduria is characterized by the excretion of C6 —C10 ω-dicarboxylic acids and by nonketotic hypoglycemia, and is caused by a lack of mitochondrial medium-chain acyl CoA dehydrogenase. Refsum disease is a rare neurologic disorder caused by a metabolic defect that results in the accumulation of phytanic acid, which is found in dairy products and ruminant fat and meat. Phytanic acid is thought to have pathologic effects on membrane function, protein prenylation, and gene expression. Zellweger (cerebrohepatorenal) syndrome occurs in individuals with a rare inherited absence of peroxisomes in all tissues. They accumulate C26 —C38 poly enoicacids in brain tissue and also exhibit a generalized loss of peroxisomal functions. The disease causes severe neurologic symptoms, and most patients die in the first year of life.

اخر الاخبار

اخبار العتبة العباسية المقدسة

أهالي كربلاء يحيون اليوم السابع لذكرى شهادة الإمام الحسين (عليه السلام)

مشروع أمير القراء الوطني يشهد مشاركة أكثر من 100 طالبٍ في مرحلته الثالثة

قسم الشؤون الفكرية يصدر دليلًا يوثّق القصص الفائزة في مسابقة فتوى الدفاع المقدسة

قسم الشؤون الفكرية يعلن عن توفير عددٍ من المكتبات الإلكترونية المتخصّصة للمؤسّسات الدينية والمكتبات العامّة

المزيد

الآخبار الصحية

نصائح عملية للحد من التعرق المفرط في الصيف

أطباء يحذرون: مكمل غذائي شائع قد يدمر الجهاز العصبي

تحذير طبي من تأثير جانبي "غير متوقع" لحقن التخسيس

حمامات الثلج قد تكون خطيرة وأحيانا مميتة.. ما السبب؟

المزيد

الآخبار التكنلوجية

أكبر جدارية شمسية في العالم تدخل موسوعة غينيس.. مشروع ضخم

تسارع دوران الأرض يثير القلق.. وتحذيرات من "كوارث محتملة" ؟

"سوذبيز" تطرح أكبر قطعة من المريخ على الأرض في مزاد علني

أوبن أيه آي تعتزم إطلاق متصفح إنترنت يعمل بالذكاء الاصطناعي

المزيد

مواضيع ذات صلة

Ketogenesis Occurs When There is a High Rate of Fatty Acid Oxidation in The Liver

β -Oxidation of Fatty Acids Involves Successive Cleavage with Release OF Acetyl-CoA

Oxidation of Fatty Acids Occurs in Mitochondria

Amphipathic Lipids Self - Orient at Oil: Water Interfaces

Lipid Peroxidation is A Source of Free Radicals

Steroids Play Many Physiologically Important Roles

Glycolipids (Glycosphingolipids) are Important in Nerve Tissue & in the Cell Membrane

Phospholipids are The Main Lipid Constituents of Membranes

Fatty Acids are Aliphatic Carboxylic Acids

The Pentose Phosphate Pathway & Other Pathways of Hexose Metabolism: clinical aspects

Galactose is Needed for the Synthesis of Lactose, Glycolipids, Proteoglycans, & Glycoproteins

Ingestion of Large Quantities of Fructose has Profound Metabolic Consequences