Rabbit LIAS Polyclonal Antibody | anti-LIAS antibody

LIAS Antibody (C-Term)

Cat. Num. AAA9217361

• Gene Names: LIAS; LS; LAS; LIP1; PDHLD; HGCLAS; HUSSY-01
• Reactivity: Mouse, Rat; Predicted: Bovine
• Applications: Western Blot, ELISA
• Synonyms: LIAS; Polyclonal Antibody; LIAS Antibody (C-Term); Lipoyl synthase; mitochondrial {ECO:0000255|HAMAP-Rule:MF_03123}; 2.8.1.8 {ECO:0000255|HAMAP-Rule:MF_03123}; Lipoate synthase {ECO:0000255|HAMAP-Rule:MF_03123}; LS {ECO:0000255|HAMAP-Rule:MF_03123}; Lip-syn {ECO:0000255|HAMAP-Rule:MF_03123}; Lipoic acid synthase {ECO:0000255|HAMAP-Rule:MF_03123}; LIAS {ECO:0000255|HAMAP-Rule:MF_03123}; LAS; Purified Rabbit Polyclonal Antibody (Pab); anti-LIAS antibody

• Host: Rabbit
• Reactivity: Mouse, Rat; Predicted: Bovine
• Clonality: Polyclonal
• Isotype: Ig
• Specificity: This LIAS antibody is generated from a rabbit immunized with a KLH conjugated synthetic peptide between 298-330 amino acids from human LIAS.
• Form/Format: Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.
• Concentration: 1.35 (varies by lot)
• Sequence Length: 372

• Applicable Applications for anti-LIAS antibody: Western Blot (WB), ELISA (EIA)
• Application Notes: WB~~1:2000
• Cellular Location: Mitochondrion {ECO:0000255|HAMAP- Rule:MF_03123}
• Antigen Type: Synthesized Peptide
• Preparation and Storage: Maintain refrigerated at 2-8 degree C for up to 2 weeks. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Western Blot (WB)

(All lanes : Anti-LIAS Antibody (C-Term) at 1:2000 dilutionLane 1: mouse heart lysateLane 2: rat heart lysateLysates/proteins at 20 ug per lane. SecondaryGoat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution. Predicted band size : 42 kDaBlocking/Dilution buffer: 5% NFDM/TBST.)

Related Product Information for anti-LIAS antibody

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.

Product Categories/Family for anti-LIAS antibody

Signal Transduction

References

Ota T.,et al.Nat. Genet. 36:40-45(2004). Hillier L.W.,et al.Nature 434:724-731(2005). Mural R.J.,et al.Submitted (JUL-2005) to the EMBL/GenBank/DDBJ databases. Stanchi F.,et al.Yeast 18:69-80(2001). Mayr J.A.,et al.Am. J. Hum. Genet. 89:792-797(2011).

NCBI and Uniprot Product Information

• NCBI GI #: 206729917
• NCBI GeneID: 11019
• UniProt Accession #: O43766; Q8IV62; A8K873; C9JCF6
• Molecular Weight: 41911 Da
• NCBI Official Full Name: Lipoyl synthase, mitochondrial
• NCBI Official Synonym Full Names: lipoic acid synthetase
• NCBI Official Symbol: LIAS
• NCBI Official Synonym Symbols: LS; LAS; LIP1; PDHLD; HGCLAS; HUSSY-01
• NCBI Protein Information: lipoyl synthase, mitochondrial
• UniProt Protein Name: Lipoyl synthase, mitochondrial
• Protein Family: Sensor histidine kinase
• UniProt Gene Name: LIAS
• UniProt Synonym Gene Names: LS; Lip-syn
• UniProt Entry Name: LIAS_HUMAN

NCBI Description

The protein encoded by this gene belongs to the biotin and lipoic acid synthetases family. It localizes in mitochondrion and plays an important role in alpha-(+)-lipoic acid synthesis. It may also function in the sulfur insertion chemistry in lipoate biosynthesis. Alternative splicing occurs at this locus and two transcript variants encoding distinct isoforms have been identified. [provided by RefSeq, Jul 2008]

Uniprot Description

LIAS: 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. Defects in LIAS are a cause of pyruvate dehydrogenase lipoic acid synthetase deficiency (PDHLD). PDHLD is an enzymatic defect resulting in an autosomal recessive disorder of mitochondrial metabolism. It is characterized by early-onset lactic acidosis, severe encephalomyopathy, and a pyruvate oxidation defect. Affected individuals have neonatal-onset epilepsy, poor growth, psychomotor retardation, muscular hypotonia, lactic acidosis, and elevated glycine concentration in plasma and urine. Belongs to the radical SAM superfamily. Lipoyl synthase family.

Protein type: Cofactor and Vitamin Metabolism - lipoic acid; Transferase; EC 2.8.1.8; Mitochondrial

Chromosomal Location of Human Ortholog: 4p14

Cellular Component: mitochondrial matrix; mitochondrion

Molecular Function: lipoate synthase activity

Biological Process: glyoxylate metabolic process; inflammatory response; lipoate biosynthetic process; protein lipoylation; response to lipopolysaccharide; response to oxidative stress

Disease: Pyruvate Dehydrogenase Lipoic Acid Synthetase Deficiency