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Mouse Anti-LIAS Recombinant Antibody (CBYJL-1642) (CBMAB-L1500-YJ)

Provided herein is a Mouse monoclonal antibody, which binds to Lipoic Acid Synthetase (LIAS). The antibody can be used for immunoassay techniques, such as ELISA, WB.
See all LIAS antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBYJL-1642
Antibody Isotype
IgG2a, κ
Application
ELISA, WB

Basic Information

Immunogen
LIAS (NP_006850, 273 a.a. ~ 373 a.a) partial recombinant protein with GST tag.
Specificity
Human
Antibody Isotype
IgG2a, κ
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!]

Buffer
PBS, pH 7.4
Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.
Epitope
aa 273-373

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. LIAS may also function in the sulfur insertion chemistry in lipoate biosynthesis. Alternative splicing occurs at this locus and two transcript variants encoding distinct isoforms have been identified. Diseases associated with LIAS include Hyperglycinemia, Lactic Acidosis, And Seizures and Lipoic Acid Synthetase Deficiency. Among its related pathways are Metabolism and Glyoxylate metabolism and glycine degradation. Gene Ontology (GO) annotations related to this gene include 4 iron, 4 sulfur cluster binding and lipoate synthase activity. LIAS 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.
Entrez Gene ID
11019
UniProt ID
O43766
Alternative Names
LS; LAS; LIP1; PDHLD; HGCLAS; HUSSY-01
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.
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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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