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LIAS Antibody (PACO27953)

SKU:
PACO27953
Product Type:
Antibody
Reactivity:
Human
Host Species:
Rabbit
Isotype:
IgG
Applications:
ELISA
WB
IHC
Antibody Type:
Polyclonal Antibody
Conjugation:
Unconjugated
  • LIAS Antibody PACO27953
  • LIAS Antibody PACO27953
$407

Description

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LIAS Antibody (PACO27953)

The LIAS Antibody (PAC027953) is a polyclonal antibody specifically designed for research involving LIAS, a protein involved in the biogenesis of iron-sulfur clusters. The antibody, produced in rabbits, exhibits high reactivity with samples from various species, including human, and has been validated for use in Western blot applications.LIAS is a crucial enzyme in the pathways responsible for synthesizing iron-sulfur clusters, essential for various biological processes such as electron transport and DNA repair. Dysregulation of LIAS has been linked to diseases like Friedreich's ataxia and multiple mitochondrial dysfunctions syndrome.

Studying the expression and activity of LIAS using this antibody can provide insights into the roles of iron-sulfur clusters in cellular function and disease pathogenesis.This LIAS Antibody is a valuable tool for researchers in the fields of biochemistry, cell biology, and genetics, allowing for the detection and analysis of LIAS protein in different cell types and tissues. Its application can contribute to advancements in understanding iron metabolism, mitochondrial function, and the molecular basis of iron-sulfur cluster disorders.

Antibody Name:LIAS Antibody (PACO27953)
Antibody SKU:PACO27953
Size:50ug
Host Species:Rabbit
Tested Applications:ELISA, WB, IHC
Recommended Dilutions:ELISA:1:2000-1:10000, WB:1:500-1:5000, IHC:1:20-1:200
Species Reactivity:Human
Immunogen:Recombinant Human Lipoyl synthase, mitochondrial protein (28-372AA)
Form:Liquid
Storage Buffer:Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Purification Method:>95%, Protein G purified
Clonality:Polyclonal
Isotype:IgG
Conjugate:Non-conjugated
Product ImageWestern blot
All lanes: LIAS antibody at 2µg/ml
Lane 1: MCF-7 whole cell lysate
Lane 2: K562 whole cell lysate
Secondary
Goat polyclonal to rabbit IgG at 1/10000 dilution
Predicted band size: 42, 37, 38 kDa
Observed band size: 42 kDa
.
Product ImageImmunohistochemistry of paraffin-embedded human small intestine tissue using PACO27953 at dilution of 1:100.
Background: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.
Synonyms:Lipoyl synthase, mitochondrial (Lipoate synthase) (LS) (Lip-syn) (Lipoic acid, synthase), LIAS, LAS
UniProt Protein Function: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. Defects in LIAS are a cause of pyruvate dehydrogenase lipoic acid synthetase deficiency (PDHLD). PDHLD is 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. Belongs to the radical SAM superfamily. Lipoyl synthase family.Protein type: Cofactor and Vitamin Metabolism - lipoic acid; Transferase; EC 2.8.1.8; MitochondrialChromosomal Location of Human Ortholog: 4p14Cellular Component: mitochondrial matrix; mitochondrionMolecular Function: lipoate synthase activityBiological Process: glyoxylate metabolic process; inflammatory response; lipoate biosynthetic process; protein lipoylation; response to lipopolysaccharide; response to oxidative stressDisease: Pyruvate Dehydrogenase Lipoic Acid Synthetase Deficiency
UniProt Protein Details:
NCBI Summary:The protein encoded by this gene 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. Alternative splicing occurs at this locus and two transcript variants encoding distinct isoforms have been identified. [provided by RefSeq, Jul 2008]
UniProt Code:O43766
NCBI GenInfo Identifier:206729917
NCBI Gene ID:11019
NCBI Accession:O43766.3
UniProt Secondary Accession:O43766,Q8IV62, A8K873, C9JCF6
UniProt Related Accession:O43766
Molecular Weight:41911 Da
NCBI Full Name:Lipoyl synthase, mitochondrial
NCBI Synonym Full Names:lipoic acid synthetase
NCBI Official Symbol:LIAS  
NCBI Official Synonym Symbols:LS; LAS; LIP1; PDHLD; HGCLAS; HUSSY-01  
NCBI Protein Information:lipoyl synthase, mitochondrial
UniProt Protein Name:Lipoyl synthase, mitochondrial
UniProt Synonym Protein Names:Lipoate synthase
Protein Family:Sensor histidine kinase
UniProt Gene Name:LIAS  
UniProt Entry Name:LIAS_HUMAN
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