Abcam, ab246917, Anti-LIAS antibody

Size: 100µL
Rabbit Polyclonal LIAS antibody. Suitable for IHC-P, ICC/IF, WB and reacts with Mouse, Human, Rat samples. Cited in 5 publications. Immunogen corresponding to Recombinant Fragment Protein within Human LIAS aa 200-350.
Key facts
Host species:Rabbit,
Clonality:Polyclonal,
Isotype:IgG,
Carrier free:No,
Reacts with:Mouse, Rat, Human,
Applications:ICC/IF, WB, IHC-PSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:Recombinant Fragment Protein within Human LIAS aa 200-350. The exact immunogen used to generate this antibody is proprietary information.O43766

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Immunogen, Storage buffer-pH: 7.2Preservative: 0.02% Sodium azideConstituents: PBS, 40% Glycerol (glycerin, glycerine), Shipped at conditions-Blue Ice, Appropriate short-term storage duration-1-2 weeks, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze / thaw cycle

Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
The biological target LIAS also known as lipoic acid synthase plays an important role in the synthesis of lipoic acid. LIAS is an enzyme with a molecular mass of approximately 40 kDa and it is present in many tissues showing high expression in liver and kidney. It functions as a homodimer in the mitochondria indicating its role as an important player in mitochondrial processes. The enzyme catalyzes the final step in the synthesis of lipoic acid by attaching sulfur atoms to octanoyl groups attached to protein domains.
Biological function summary
LIAS contributes to the activation of metabolic complexes by providing protein-bound lipoic acid. It integrates into multiprotein complexes facilitating their function. This lipoic acid acts as a cofactor for key mitochondrial enzyme complexes such as pyruvate dehydrogenase and α-ketoglutarate dehydrogenase which are important for energy production. These complexes are essential for the conversion of nutrients into usable energy highlighting the biochemical importance of LIAS in cellular respiration and metabolic regulation.
Pathways
Lipoic acid synthase is fundamental for mitochondrial energy metabolism and redox regulation pathways. It participates in the tricarboxylic acid cycle where it influences the activity of related enzymes. Through this activity LIAS interacts indirectly with proteins such as pyruvate dehydrogenase and dihydrolipoamide dehydrogenase both of which play a significant role in maintaining cellular energy balance. The efficient functioning of these pathways is important for cellular survival and homeostasis.
Mutations in LIAS have been linked to rare metabolic disorders like multiple mitochondrial dysfunctions syndrome. These mutations can lead to severe energy production deficits due to impaired enzyme complex assembly. In these conditions LIAS's connection to lipoic acid-dependent proteins like pyruvate dehydrogenase impacts disease progression further demonstrating its role in mitochondrial health. On a broader scale alterations in LIAS function might also relate to disorders involving energy metabolism such as neurodegenerative diseases.