LIAS Antibody

Datasheet

Code	CSB-PA012927ZA01HU
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Target Background

Product Details

Full Product Name

Rabbit anti-Homo sapiens (Human) LIAS Polyclonal antibody

Uniprot No.

O43766

Target Names

LIAS

Alternative Names

LIAS antibody; LAS antibody; HUSSY-01Lipoyl synthase antibody; mitochondrial antibody; EC 2.8.1.8 antibody; Lipoate synthase antibody; LS antibody; Lip-syn antibody; Lipoic acid synthase antibody

Raised in

Rabbit

Species Reactivity

Homo sapiens

Immunogen

Recombinant Homo sapiens LIAS protein

Immunogen Species

Homo sapiens (Human)

Conjugate

Non-conjugated

Clonality

Polyclonal

Isotype

IgG

Purification Method

Antigen Affinity Purified

Concentration

It differs from different batches. Please contact us to confirm it.

Buffer

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4

Form

Liquid

Tested Applications

ELISA, WB (ensure identification of antigen)

Protocols

ELISA Protocol

Troubleshooting and FAQs

Antibody FAQs

Storage

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Value-added Deliverables

① 200ug * antigen (positive control);
② 1ml * Pre-immune serum (negative control);

Quality Guarantee

① Antibody purity can be guaranteed above 90% by SDS-PAGE detection;
② ELISA titer can be guaranteed 1: 64,000;
③ WB validation with antigen can be guaranteed positive;

Lead Time

Made-to-order (12-14 weeks)

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Target Background

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.

Gene References into Functions

this study used a bioinformatics approach to predict its structure. . A homology model for LIAS protein was generated using X-ray crystallographic structure of Thermosynechococcus elogatsu. The active site of LIAS protein was mapped and docked with S-Adenosyl Methionine PMID: 27717843
oxoglutarate dehydrogenase (OGDH) and lipoic acid synthase (LIAS), which when mutated stabilize HIF1alpha in a non-hydroxylated form. PMID: 27923773
heterozygous mutations (c.738-2A>G and c.929T>C (p.Met310Thr)) in LIAS. PMID: 26108146
Patients with LIAS nonketotic hyperglycinemia varied in disease severity and cortical involvement. PMID: 24334290
We identified the homozygous mutation c.746G>A (p.Arg249His) in LIAS in an individual with neonatal-onset epilepsy, muscular hypotonia, lactic acidosis, and elevated glycine concentration in plasma and urine PMID: 22152680
Lipoic acid synthetase deficiency results in an overall disturbance in the antioxidant defense network, leading to increased inflammation, insulin resistance, and mitochondrial dysfunction. PMID: 19074983

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Involvement in disease

Hyperglycinemia, lactic acidosis, and seizures (HGCLAS)

Subcellular Location

Mitochondrion.

Protein Families

Radical SAM superfamily, Lipoyl synthase family

Database Links

HGNC: 16429

OMIM: 607031

KEGG: hsa:11019

STRING: 9606.ENSP00000261434

UniGene: Hs.550502

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Protein Service

Gene Synthesis Service

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LIAS Antibody

Product Details

Related Products

Customer Reviews and Q&A

Target Background