Rabbit LIAS Polyclonal Antibody | anti-LIAS antibody

LIAS Rabbit Polyclonal

Cat. Num. AAA7601005

• Gene Names: LIAS; LS; LAS; LIP1; PDHLD; HGCLAS; HUSSY-01
• Reactivity: Human, Mouse, Rat
• Applications: ELISA, Western Blot, Immunohistochemistry
• Purity: >=95% as determined by SDS-PAGE; Immunogen Affinity Purified
• Synonyms: LIAS; Polyclonal Antibody; LIAS Rabbit Polyclonal; anti-LIAS antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: >=95% as determined by SDS-PAGE; Immunogen Affinity Purified
• Form/Format: Liquid
• Sequence Length: 100

• Applicable Applications for anti-LIAS antibody: ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
• Immunogen: Lipoic acid synthetase
• Preparation and Storage: PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded human lymphoma using MBS7601005 (LIAS antibody) at dilution of 1:100)

Western Blot (WB)

(human brain tissue were subjected to SDS PAGE followed by western blot with MBS7601005 (LIAS antibody) at dilution of 1:500)

NCBI and Uniprot Product Information

• NCBI GI #: 515869855
• NCBI GeneID: 11019
• NCBI Accession #: NP_001265521.1
• NCBI GenBank Nucleotide #: NM_001278592.1
• Molecular Weight: 37,137 Da
• NCBI Official Full Name: lipoyl synthase, mitochondrial isoform 5
• 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; Mitochondrial; EC 2.8.1.8; Transferase

Chromosomal Location of Human Ortholog: 4p14

Cellular Component: mitochondrial matrix; mitochondrion

Molecular Function: 4 iron, 4 sulfur cluster binding; lipoate synthase activity; metal ion binding

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

Disease: Pyruvate Dehydrogenase Lipoic Acid Synthetase Deficiency