Human Very long-chain specific acyl-CoA dehydrogenase, mitochondrial, ACADVL ELISA Kit | MyBioSource MBS9316130 product information

product summary

company name :

MyBioSource

product type :

ELISA/assay

product name :

Human Very long-chain specific acyl-CoA dehydrogenase, mitochondrial, ACADVL ELISA Kit

catalog :

MBS9316130

quantity :

48-Strip-Wells

price :

435 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS9316130

products type :

ELISA Kit

products full name :

Human Very long-chain specific acyl-CoA dehydrogenase, mitochondrial, ACADVL ELISA Kit

products short name :

acyl-Coenzyme A dehydrogenase, very long chain

products name syn :

Human Very long-chain specific acyl-CoA dehydrogenase; mitochondrial (ACADVL) ELISA kit; ACAD6; LCACD; VLCAD; ; acyl-Coenzyme A dehydrogenase; very long chain

other names :

very long-chain specific acyl-CoA dehydrogenase, mitochondrial isoform 1; Very long-chain specific acyl-CoA dehydrogenase, mitochondrial; very long-chain specific acyl-CoA dehydrogenase, mitochondrial; acyl-Coenzyme A dehydrogenase, very long chain; acyl-CoA dehydrogenase, very long chain

products gene name :

ACADVL

other gene names :

ACADVL; ACADVL; ACAD6; LCACD; VLCAD; VLCAD; VLCAD

uniprot entry name :

ACADV_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 :

Assay Type: 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/Introduction: 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 ACADVL (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 :

4557235

ncbi acc num :

NP_000009.1

ncbi gb acc num :

NM_000018.3

uniprot acc num :

P49748

ncbi mol weight :

70,390 Da

ncbi pathways :

Beta Oxidation Of Palmitoyl-CoA To Myristoyl-CoA Pathway (106124); FOXA2 And FOXA3 Transcription Factor Networks Pathway (137911); Fatty Acid Beta Oxidation Pathway (198865); Fatty Acid Degradation Pathway (82935); Fatty Acid Degradation Pathway (296); Fatty Acid Metabolism Pathway (868084); Fatty Acid Metabolism Pathway (878045); Fatty Acid, Triacylglycerol, And Ketone Body Metabolism Pathway (160977); IRE1alpha Activates Chaperones Pathway (105906); Metabolic Pathways (132956)

ncbi summary :

The protein encoded by this gene is targeted to the inner mitochondrial membrane where it catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. This acyl-Coenzyme A dehydrogenase is specific to long-chain and very-long-chain fatty acids. A deficiency in this gene product reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Jul 2008]

uniprot summary :

ACADVL: Active toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. Can accommodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons. Defects in ACADVL are the cause of acyl-CoA dehydrogenase very long chain deficiency (ACADVLD). ACADVLD is an autosomal recessive disease which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form, with early onset, high mortality, and high incidence of cardiomyopathy; a milder childhood form, with later onset, usually with hypoketotic hypoglycemia as the main presenting feature, low mortality, and rare cardiomyopathy; and an adult form, with isolated skeletal muscle involvement, rhabdomyolysis, and myoglobinuria, usually triggered by exercise or fasting. Belongs to the acyl-CoA dehydrogenase family. 2 isoforms of the human protein are produced by alternative splicing. Protein type: Mitochondrial; Lipid Metabolism - fatty acid; EC 1.3.8.9; Oxidoreductase. Chromosomal Location of Human Ortholog: 17p13.1. Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial inner membrane; cytoplasm; nucleolus; nucleus. Molecular Function: acyl-CoA dehydrogenase activity; FAD binding; long-chain-acyl-CoA dehydrogenase activity. Biological Process: cellular protein metabolic process; fatty acid beta-oxidation; unfolded protein response, activation of signaling protein activity; epithelial cell differentiation; unfolded protein response; cellular lipid metabolic process; thermoregulation; fatty acid beta-oxidation using acyl-CoA dehydrogenase; negative regulation of fatty acid oxidation; negative regulation of fatty acid biosynthetic process; energy derivation by oxidation of organic compounds. Disease: Acyl-coa Dehydrogenase, Very Long-chain, Deficiency Of

size1 :

48-Strip-Wells

price1 :

435 USD

size2 :

96-Strip-Wells

price2 :

600

more info or order :

MyBioSource product webpage