Name: LIAS Antibody (PACO27953)
SKU: PACO27953
Availability: OutOfStock

Antibody Name:	LIAS Antibody (PACO27953)
Antibody SKU:	PACO27953
Size:	50ug
Host Species:	Rabbit
Tested Applications:	ELISA, WB, IHC
Recommended Dilutions:	ELISA:1:2000-1:10000, WB:1:500-1:5000, IHC:1:20-1:200
Species Reactivity:	Human
Immunogen:	Recombinant Human Lipoyl synthase, mitochondrial protein (28-372AA)

Form:	Liquid
Storage Buffer:	Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Purification Method:	>95%, Protein G purified
Clonality:	Polyclonal
Isotype:	IgG
Conjugate:	Non-conjugated

	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 PACO27953 at dilution of 1:100.

Background:	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.
Synonyms:	Lipoyl synthase, mitochondrial (Lipoate synthase) (LS) (Lip-syn) (Lipoic acid, synthase), LIAS, LAS

UniProt Protein Function:	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; MitochondrialChromosomal Location of Human Ortholog: 4p14Cellular Component: mitochondrial matrix; mitochondrionMolecular Function: lipoate synthase activityBiological Process: glyoxylate metabolic process; inflammatory response; lipoate biosynthetic process; protein lipoylation; response to lipopolysaccharide; response to oxidative stressDisease: Pyruvate Dehydrogenase Lipoic Acid Synthetase Deficiency
UniProt Protein Details:
NCBI Summary:	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 Code:	O43766
NCBI GenInfo Identifier:	206729917
NCBI Gene ID:	11019
NCBI Accession:	O43766.3
UniProt Secondary Accession:	O43766,Q8IV62, A8K873, C9JCF6
UniProt Related Accession:	O43766
Molecular Weight:	41911 Da
NCBI Full Name:	Lipoyl synthase, mitochondrial
NCBI 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
UniProt Synonym Protein Names:	Lipoate synthase
Protein Family:	Sensor histidine kinase
UniProt Gene Name:	LIAS
UniProt Entry Name:	LIAS_HUMAN