Mouse Anti-LIAS Recombinant Antibody (CBYCL-309) (CBMAB-L0210-YC)

Provided herein is a Mouse monoclonal antibody against Human LIAS. The antibody can be used for immunoassay techniques, such as WB.
See all LIAS antibodies

Datasheet

Summary

Host Animal

Mouse

Specificity

Human

Clone

CBYCL-309

Antibody Isotype

IgG2a

Application

Basic Information

Specificity

Human

Antibody Isotype

IgG2a

Clonality

Monoclonal

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Storage

Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name

lipoic acid synthetase

Introduction

LIAS belongs to the biotin and lipoic acid synthetases family. It localizes in mitochondrion and plays an important role in alpha-(+)-lipoic acid synthesis. It may also function in the sulfur insertion chemistry in lipoate biosynthesis. Diseases associated with LIAS include Hyperglycinemia, Lactic Acidosis, And Seizures and Lipoic Acid Synthetase Deficiency.

Entrez Gene ID

11019

UniProt ID

O43766

Alternative Names

HGCLAS; HUSSY-01; LAS; LIP1; LS; PDHLD

Function

Catalyzes the radical-mediated insertion of two sulfur atoms into the C-6 and C-8 positions of the octanoyl moiety bound to the lipoyl domains of lipoate-dependent enzymes, thereby converting the octanoylated domains into lipoylated derivatives.

Biological Process

Inflammatory responseISS:UniProtKB
Lipoate biosynthetic processISS:UniProtKB
Neural tube closureIEA:Ensembl
Protein lipoylationIEA:UniProtKB-UniRule
Response to lipopolysaccharideISS:UniProtKB
Response to oxidative stressISS:UniProtKB

Cellular Location

Mitochondrion

Involvement in disease

Hyperglycinemia, lactic acidosis, and seizures (HGCLAS):
An enzymatic defect resulting in an autosomal recessive disorder of mitochondrial metabolism. It is characterized by early-onset lactic acidosis, severe encephalomyopathy, and a pyruvate oxidation defect. Affected individuals have neonatal-onset epilepsy, poor growth, psychomotor retardation, muscular hypotonia, lactic acidosis, and elevated glycine concentration in plasma and urine.

References

Bilska-Wilkosz, A. (2023). The S-adenosyl-l-methionine radical enzymes: the lipoic acid synthase and the biotin synthase. In Sulfurtransferases (pp. 177-206). Academic Press.

Hendricks, A. L., Wachnowsky, C., Fries, B., Fidai, I., & Cowan, J. A. (2021). Characterization and reconstitution of human lipoyl synthase (LIAS) supports ISCA2 and ISCU as primary cluster donors and an ordered mechanism of cluster assembly. International Journal of Molecular Sciences, 22(4), 1598.

Zhao, Y., Yan, T., Xiong, C., Chang, M., Gao, Q., Yao, S., ... & Xu, G. (2021). Overexpression of lipoic acid synthase gene alleviates diabetic nephropathy of Leprdb/db mice. BMJ Open Diabetes Research & Care, 9(1).

Cronan, J. E. (2020). Progress in the enzymology of the mitochondrial diseases of lipoic acid requiring enzymes. Frontiers in Genetics, 11, 510.

Zhao, Y., Xu, G., Li, H., Chang, M., Guan, Y., Li, Y., ... & Yao, S. (2020). Overexpression of endogenous lipoic acid synthase attenuates pulmonary fibrosis induced by crystalline silica in mice. Toxicology Letters, 323, 57-66.

Tian, S., Nakamura, J., Hiller, S., Simington, S., Holley, D. W., Mota, R., ... & Yi, X. (2020). New insights into immunomodulation via overexpressing lipoic acid synthase as a therapeutic potential to reduce atherosclerosis. Vascular pharmacology, 133, 106777.

Solmonson, A., & DeBerardinis, R. J. (2018). Lipoic acid metabolism and mitochondrial redox regulation. Journal of Biological Chemistry, 293(20), 7522-7530.

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Protocols

Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

Associated Products

Related Products

Mouse Anti-LIAS Recombinant Antibody (CBYJL-1642)

Isotype control

For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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