LIAS Antibody

Datasheet

Code	CSB-PA012927LA01HU
Size	US$166
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Image	Western 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 Immunohistochemistry of paraffin-embedded human small intestine tissue using CSB-PA012927LA01HU at dilution of 1:100
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Product Details
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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

Human

Immunogen

Recombinant Human Lipoyl synthase, mitochondrial protein (28-372AA)

Immunogen Species

Homo sapiens (Human)

Conjugate

Non-conjugated

The LIAS Antibody (Product code: CSB-PA012927LA01HU) is Non-conjugated. For LIAS Antibody with conjugates, please check the following table.

Conjugate	Product Code	Product Name	Application
HRP	CSB-PA012927LB01HU	LIAS Antibody, HRP conjugated	ELISA
FITC	CSB-PA012927LC01HU	LIAS Antibody, FITC conjugated
Biotin	CSB-PA012927LD01HU	LIAS Antibody, Biotin conjugated	ELISA

Clonality

Polyclonal

Isotype

IgG

Purification Method

>95%, Protein G 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, IHC

Recommended Dilution

Application	Recommended Dilution
WB	1:500-1:5000
IHC	1:20-1:200

Protocols

ELISA Protocol
Western Blotting(WB) Protocol
Immunohistochemistry (IHC) Protocol

Troubleshooting and FAQs

Antibody FAQs

Storage

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

Lead Time

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

Customer Reviews and Q&A

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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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