935106861
info@markelab.com
Precio
281.25€ (80 µl)
The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family, which is a family of mitochondrial flavoenzymes involved in fatty acid and branched chain amino-acid metabolism. This protein is one of the four enzymes that catalyze the initial step of mitochondrial beta-oxidation of straight-chain fatty acid. Defects in this gene are the cause of long-chain acyl-CoA dehydrogenase (LCAD) deficiency, leading to nonketotic hypoglycemia.
ACADL , also known as long-chain acyl-CoA dehydrogenase (LCAD), is a mitochondrial enzyme critical for the β-oxidation of long-chain fatty acids. It catalyzes the first step in the oxidative breakdown of long-chain fatty acyl-CoAs, facilitating their conversion into energy through the tricarboxylic acid (TCA) cycle and electron transport chain. ACADL is predominantly expressed in energy-demanding tissues such as the liver, heart, and skeletal muscle, where fatty acid metabolism is essential for maintaining energy homeostasis during fasting and sustained physical activity. Dysregulation or mutations in the ACADL gene can result in long-chain acyl-CoA dehydrogenase deficiency, a rare metabolic disorder characterized by hypoglycemia, muscle weakness, and lipid accumulation due to impaired fatty acid oxidation. ACADL also plays a role in lipid signaling and mitochondrial health, contributing to cellular responses to metabolic stress. Its enzymatic activity underscores its importance in energy metabolism, lipid regulation, and mitochondrial function, making it a key target for studies on metabolic diseases and energy balance.
Primary Antibodies
Polyclonal
Human, Mouse, Rat
Acyl-CoA Dehydrogenase, Long Chain (ACADL)
Rabbit
Unconjugated
Liquid
ELISA, WB
Purified through a protein A column, followed by peptide affinity purification.
PBS containing 0.09% sodium azide.
80 µl
400 µl
Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles.
ACADL
No
Shipped within 5-10 working days.
ACAD4,LCAD
This product is for research use only.
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Ver másACADL(Long-chain specific acyl-CoA dehydrogenase, mitochondrial) is also named as LCAD and belongs to the acyl-CoA dehyd...
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