IVD Antibody (PACO60725)
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주문- SKU:
- PACO60725
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Antibody Name: | IVD Antibody (PACO60725) |
Antibody SKU: | PACO60725 |
Size: | 50ug |
Host Species: | Rabbit |
Tested Applications: | ELISA, IHC |
Recommended Dilutions: | ELISA:1:2000-1:10000, IHC:1:500-1:1000 |
Species Reactivity: | Human |
Immunogen: | Recombinant Human Isovaleryl-CoA dehydrogenase, mitochondrial protein (139-241AA) |
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 |
IHC image of PACO60725 diluted at 1:500 and staining in paraffin-embedded human appendix tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system. |
Background: | mitochondrial matrix, isovaleryl-CoA dehydrogenase activity, branched-chain amino acid, catabolic process, leucine catabolic process |
Synonyms: | Isovaleryl-CoA dehydrogenase, mitochondrial (IVD) (EC 1.3.8.4), IVD |
UniProt Protein Function: | IVD: Defects in IVD are the cause of isovaleric acidemia (IVA). IVA is characterized by retarded psychomotor development, a peculiar odor resembling sweaty feet, an aversion to dietary protein, and pernicious vomiting, leading to acidosis and coma. The acute neonatal form leads to massive metabolic acidosis from the first days of life and rapid death. Belongs to the acyl-CoA dehydrogenase family. |
UniProt Protein Details: | Protein type:EC 1.3.8.4; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Mitochondrial; Oxidoreductase Chromosomal Location of Human Ortholog: 15q14-q15 Cellular Component: nucleoplasm; mitochondrion; mitochondrial matrix Molecular Function:isovaleryl-CoA dehydrogenase activity; FAD binding Biological Process: leucine catabolic process; branched chain family amino acid catabolic process Disease: Isovaleric Acidemia |
NCBI Summary: | 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. [provided by RefSeq, Apr 2009] |
UniProt Code: | P26440 |
NCBI GenInfo Identifier: | 226958414 |
NCBI Gene ID: | 3712 |
NCBI Accession: | NP_001152980.1 |
UniProt Secondary Accession: | P26440,Q53XZ9, Q96AF6, B2RCV5, B3KVI7, J3KR54, |
UniProt Related Accession: | P26440 |
Molecular Weight: | 46,319 Da |
NCBI Full Name: | isovaleryl-CoA dehydrogenase, mitochondrial isoform 2 |
NCBI Synonym Full Names: | isovaleryl-CoA dehydrogenase |
NCBI Official Symbol: | IVD |
NCBI Official Synonym Symbols: | ACAD2 |
NCBI Protein Information: | isovaleryl-CoA dehydrogenase, mitochondrial; isovaleryl CoA dehydrogenase; isovaleryl Coenzyme A dehydrogenase |
UniProt Protein Name: | Isovaleryl-CoA dehydrogenase, mitochondrial |
Protein Family: | Isovaleryl-CoA dehydrogenase |
UniProt Gene Name: | IVD |
UniProt Entry Name: | IVD_HUMAN |
Antibodies | ELISA Kits |
Anti-IVD Antibody (CAB15281) | Human IVD (Isovaleryl-CoA dehydrogenase) ELISA Kit (HUFI03782) |
IVD Antibody (PACO10021) |
Secondary Antibody |
Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014) |
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Anti-FITC Goat Anti-Rabbit IgG (H+L) Antibody (CABS011) |
Anti-HRP-conjugated Beta Actin Antibody (CABC028) |