product summary
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company name :
MyBioSource
product type :
ELISA/assay
product name :
Human Isovaleryl-CoA dehydrogenase, mitochondrial, IVD ELISA Kit
catalog :
MBS9327903
quantity :
48-Strip-Wells
price :
470 USD
more info or order :
MyBioSource product webpage
product information
catalog number :
MBS9327903
products type :
ELISA Kit
products full name :
Human Isovaleryl-CoA dehydrogenase, mitochondrial, IVD ELISA Kit
products short name :
[isovaleryl Coenzyme A dehydrogenase]
products name syn :
[Human Isovaleryl-CoA dehydrogenase; mitochondrial (IVD) ELISA kit; ACAD2; FLJ12715; FLJ34849; ; isovaleryl Coenzyme A dehydrogenase]
other names :
[isovaleryl-CoA dehydrogenase, mitochondrial isoform 2; Isovaleryl-CoA dehydrogenase, mitochondrial; isovaleryl-CoA dehydrogenase, mitochondrial; isovaleryl CoA dehydrogenase; isovaleryl Coenzyme A dehydrogenase; isovaleryl-CoA dehydrogenase]
products gene name :
[IVD]
other gene names :
[IVD; IVD; ACAD2; IVD]
uniprot entry name :
IVD_HUMAN
reactivity :
Human
specificity :
No significant cross-reactivity or interference between this analyte and analogues is observed.
storage stability :
Store all reagents at 2-8 degree C
other info1 :
Samples: undiluted original Human body fluids, tissue homogenates, secretions or feces samples. Assay Type: Quantitative Sandwich. Detection Range: 0.625 ng/ml - 20 ng/mL. Sensitivity: 0.1 ng/mL
other info2 :
Intra-assay Precision: Intra-assay CV (%) is less than 15%. Inter-assay Precision: Inter-assay CV (%) is less than 15%. [CV(%) = SD/mean ×100].
products description :
Background: This Quantitative Sandwich ELISA kit is only for in vitro research use only, not for drug, household, therapeutic or diagnostic applications! This kit is intended to be used for determination the level of IVD (hereafter termed "analyte") in undiluted original Human body fluids, tissue homogenates, secretions or feces samples. This kit is NOT suitable for assaying non-biological sources of substances.
ncbi gi num :
226958414
ncbi acc num :
NP_001152980.1
ncbi gb acc num :
NM_001159508.1
uniprot acc num :
P26440
ncbi mol weight :
46,319 Da
ncbi pathways :
Branched-chain Amino Acid Catabolism Pathway (106179); Leucine Degradation, Leucine = Acetoacetate + Acetyl-CoA Pathway (413354); Leucine Degradation, Leucine = Acetoacetate + Acetyl-CoA Pathway (468229); Metabolism Pathway (477135); Metabolism Of Amino Acids And Derivatives Pathway (106169); Valine, Leucine And Isoleucine Degradation Pathway (82952); Valine, Leucine And Isoleucine Degradation Pathway (316); Leucine Degradation I Pathway (142359)
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 summary :
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. 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
size1 :
48-Strip-Wells
price1 :
470 USD
size2 :
96-Strip-Wells
price2 :
680
size3 :
5x96-Strip-Wells
price3 :
3100
size4 :
10x96-Strip-Wells
price4 :
6095
more info or order :
MyBioSource product webpage
company information
MyBioSource
P.O. Box 153308
San Diego, CA 92195-3308
sales@mybiosource.com
https://www.mybiosource.com
1-888-627-0165
headquarters: USA
MyBioSource, LLC was orginally founded in Vancouver by three enthusiastic scientists who are passionate about providing the world with the best reagents available. Together, they form a company with a big vision known as MyBioSource. MyBioSource is now located in San Diego, California, USA.

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