Goat anti-VLCAD Antibody | MyBioSource MBS422310 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

Goat anti-VLCAD Antibody

catalog :

MBS422310

quantity :

0.1 mg

price :

300 USD

clonality :

polyclonal

host :

goat

conjugate :

nonconjugated

reactivity :

human

application :

western blot, enzyme immunoassay

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS422310

products type :

Antibody

products full name :

Goat anti-VLCAD Antibody

products short name :

VLCAD

products name syn :

ACADVL; acyl-Coenzyme A dehydrogenase, very long chain; ACAD6; LCACD; VLCAD; ACAD6 antibody; acyl-Coenzyme A dehydrogenase; very long chain antibody; LCACD antibody; VLCAD antibody; ACADVL antibody

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-CoA dehydrogenase, very long chain

products gene name :

ACADVL

products gene name syn :

VLCAD

other gene names :

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

uniprot entry name :

ACADV_HUMAN

clonality :

Polyclonal

host :

Goat

reactivity :

Tested: Human; Expected from sequence similarity: Human

sequence length :

655

sequence :

DKSDSHPSDALTRK

purity :

Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide

form :

Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.

concentration :

100ug specific antibody in 200ul

storage stability :

Aliquot and store at -20 degree C. Minimize freezing and thawing.

tested application :

Peptide ELISA (EIA), Western Blot (WB)

app notes :

Peptide ELISA: Antibody detection limit dilution 1: 2000. Western Blot: Approx 68kDa band observed in Human Heart lysates (calculated MW of 68.1kDa according to NP_001029031.1). Recommended concentration: 1-3ug/ml.

other info1 :

Immunogen: Peptide with sequence DKSDSHPSDALTRK-C, from the N-Terminus (near) of the protein sequence according to NP_000009.1; NP_001029031.1. Epitope: N-Terminus (near)

other info2 :

Note: This antibody is expected to recognize both reported isoforms (NP_000009.1; NP_001029031.1).

ncbi gi num :

4557235

ncbi acc num :

NP_000009.1

ncbi gb acc num :

NM_000018.3

ncbi mol weight :

72,927 Da

ncbi pathways :

Beta Oxidation Of Palmitoyl-CoA To Myristoyl-CoA Pathway (1270017); 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 (1270010); IRE1alpha Activates Chaperones Pathway (1268759); 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: EC 1.3.8.9; Mitochondrial; Lipid Metabolism - fatty acid; Oxidoreductase. Chromosomal Location of Human Ortholog: 17p13.1. Cellular Component: cytoplasm; mitochondrial inner membrane; mitochondrial matrix; mitochondrion; nucleolus; nucleus. Molecular Function: acyl-CoA binding; acyl-CoA dehydrogenase activity; electron carrier activity; FAD binding; long-chain-acyl-CoA dehydrogenase activity; very-long-chain-acyl-CoA dehydrogenase activity. Biological Process: energy derivation by oxidation of organic compounds; epithelial cell differentiation; fatty acid beta-oxidation; fatty acid beta-oxidation using acyl-CoA dehydrogenase; lipid homeostasis; negative regulation of fatty acid biosynthetic process; negative regulation of fatty acid oxidation; thermoregulation; very-long-chain fatty acid catabolic process. Disease: Acyl-coa Dehydrogenase, Very Long-chain, Deficiency Of

size1 :

0.1 mg

price1 :

300 USD

more info or order :

MyBioSource product webpage