LIAS Antibody

Code: CSB-PA012927GA01HU

Rate （N/A）
Review （0）

Size:

100μl

US$600

Quantity:

Species Reactivity: Human,Mouse,Rat

Raised in: Rabbit

Application: ELISA,WB,IHC

Send an Inquiry

Start an on-line Chat

Online ordering is currently available for U.S. customers only. For orders outside the U.S., please kindly submit an inquiry or start a chat with us.

Product Details
Related Products
Customer Reviews and Q&A
Target Background

Product Details

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,Mouse,Rat

Immunogen

Human LIAS

Immunogen Species

Homo sapiens (Human)

Isotype

IgG

Purification Method

Antigen Affinity Purified

Concentration

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

Buffer

PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.

Tested Applications

ELISA,WB,IHC

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.
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.

Usage

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

Datasheet & COA

Antibody FAQs

Customer Reviews and Q&A

■ Customer Reviews

Submit a Review here

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

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