LIAS Antibody

Code: CSB-PA012927ZA01HU
Size:
0.1ml
0.1ml1ml
US$Enquire
Quantity:
Species Reactivity: Homo sapiens
Raised in: Rabbit
Application: ELISA, WB (ensure identification of antigen)
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Product Details

Uniprot NO.
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
Conjugate
Non-conjugated
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
Tested Applications
ELISA, WB (ensure identification of antigen)
Lead Time
Made-to-order (12-14 weeks)
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
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;
Datasheet & COA

Customer Reviews and Q&A

 Customer Reviews

Target Background

Function(From Uniprot)
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
  1. 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
  2. oxoglutarate dehydrogenase (OGDH) and lipoic acid synthase (LIAS), which when mutated stabilize HIF1alpha in a non-hydroxylated form. PMID:27923773
  3. heterozygous mutations (c.738-2A>G and c.929T>C (p.Met310Thr)) in LIAS. PMID:26108146
  4. Patients with LIAS nonketotic hyperglycinemia varied in disease severity and cortical involvement. PMID:24334290
  5. 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
  6. 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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Subcellular Location
Mitochondrion.
Protein Families
Radical SAM superfamily, Lipoyl synthase family
Database Links

HGNC: 16429

UNIGENE: Hs.550502

KEGG: hsa:11019

STRING: 9606.ENSP00000261434

OMIM: 607031

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