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.

Usage

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

Customer Reviews and Q&A

■ Customer Reviews

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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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301-363-4651 (Available 9 a.m. to 5 p.m. CST from Monday to Friday)

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Address

7505 Fannin St., Ste 610, Room 7 (CUBIO Innovation Center), Houston, TX 77054, USA

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