Western Blot (WB)
(Lanes:1: Normal controls Normal enzyme expression2: Normal controls Normal enzyme expression3: Positive mutants Defective enzyme expression4: Positive mutants Defective enzyme expression5: Positive mutants Defective enzyme expressionPrimary Antibody Dilution:1:2000Secondary Antibody:Secondary Antibody Dilution:1:1000Gene Name:ACADVLSubmitted by:David Lombert, Medical College of Wisconsin)
Rabbit ACADVL Polyclonal Antibody | anti-ACADVL antibody
ACADVL antibody - C-terminal region
Western Blot (WB)
(Lanes:1: Normal controls Normal enzyme expression2: Normal controls Normal enzyme expression3: Positive mutants Defective enzyme expression4: Positive mutants Defective enzyme expression5: Positive mutants Defective enzyme expressionPrimary Antibody Dilution:1:2000Secondary Antibody:Secondary Antibody Dilution:1:1000Gene Name:ACADVLSubmitted by:David Lombert, Medical College of Wisconsin)
Western Blot (WB)
(Lanes:1: Normal controls Normal enzyme expression2: Normal controls Normal enzyme expression3: Positive mutants Defective enzyme expression4: Positive mutants Defective enzyme expression5: Positive mutants Defective enzyme expressionPrimary Antibody Dilution:1:2000Secondary Antibody:Secondary Antibody Dilution:1:1000Gene Name:ACADVLSubmitted by:David Lombert, Medical College of Wisconsin)
Western Blot (WB)
(WB Suggested Anti-ACADVL Antibody Titration: 0.2-1 ug/mlELISA Titer: 1:1562500Positive Control: ACHN cell lysateACADVL is supported by BioGPS gene expression data to be expressed in ACHN)
Western Blot (WB)
(WB Suggested Anti-ACADVL Antibody Titration: 0.2-1 ug/mlELISA Titer: 1:1562500Positive Control: ACHN cell lysateACADVL is supported by BioGPS gene expression data to be expressed in ACHN)
Target Description: ACADVL 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 ACADVL protein reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy.
NCBI and Uniprot Product Information
NCBI Description
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 Description
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: Oxidoreductase; EC 1.3.8.9; Lipid Metabolism - fatty acid; Mitochondrial
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: unfolded protein response, activation of signaling protein activity; cellular protein metabolic process; fatty acid beta-oxidation; epithelial cell differentiation; unfolded protein response; cellular lipid metabolic process; thermoregulation; fatty acid beta-oxidation using acyl-CoA dehydrogenase; negative regulation of fatty acid biosynthetic process; negative regulation of fatty acid oxidation; energy derivation by oxidation of organic compounds
Disease: Acyl-coa Dehydrogenase, Very Long-chain, Deficiency Of
Research Articles on ACADVL
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Product Notes
The ACADVL acadvl (Catalog #AAA3211696) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The ACADVL antibody - C-terminal region reacts with Cow, Dog, Guinea Pig, Horse, Human, Mouse, Rabbit, Rat, Zebrafish and may cross-react with other species as described in the data sheet. AAA Biotech's ACADVL can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). Researchers should empirically determine the suitability of the ACADVL acadvl for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. The amino acid sequence is listed below: Synthetic peptide located within the following region: IDLYAMVVVL SRASRSLSEG HPTAQHEKML CDTWCIEAAA RIREGMAALQ. It is sometimes possible for the material contained within the vial of "ACADVL, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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