IVD Antibody

Datasheet
Code CSB-PA011921GA01HU
Product Type Purified Rabbit Anti human PolyClonal Antibody
Size US$685
Uniprot No. P26440
Immunogen Human IVD
Raised in Rabbit
Species Reactivity Human,Mouse,Rat
Tested Applications ELISA,IHC
Storage Buffer PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
Purification Method Antigen Affinity Purified
Isotype IgG
Alias isovaleryl Coenzyme A dehydrogenase;IVD;ACAD2 ;
Immunogen Species Homo sapiens (Human)
Protocols ELISA Protocol
Immunohistochemistry (IHC) Protocol
Target Names IVD
Storage Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Target Details Isovaleryl-CoA dehydrogenase (IVD) is a mitochondrial matrix enzyme that catalyzes the third step in leucine catabolism. The genetic deficiency of IVD results in an accumulation of isovaleric acid, which is toxic to the central nervous system and leads to isovaleric acidemia. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.
HGNC 6186
RGD 2936
MGI 1929242
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Involvement in disease Isovaleric acidemia (IVA)
Subcellular Location Mitochondrion matrix
Protein Families Acyl-CoA dehydrogenase family
Database Links

HGNC: 6186

OMIM: 243500

KEGG: hsa:3712

STRING: 9606.ENSP00000418397

UniGene: Hs.513646

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