How is the antibody shipped, and does this affect its stability?

The antibody is typically shipped on ice packs or dry ice to maintain its stability during transit. Recombinant antibodies are generally quite stable. While they are stable at ambient temperatures for short periods (e.g., during shipping), long-term stability is best maintained under refrigerated or frozen conditions as recommended. The shipping method is designed to ensure the product arrives in optimal condition, and it does not negatively impact the antibody's performance when stored correctly upon arrival.

How is the specificity of the Mouse Anti-ACADVL Recombinant Antibody (V2-610911) confirmed?

The specificity of this recombinant antibody is typically confirmed through rigorous validation processes including Western Blot analysis against cell lysates or tissue extracts known to express ACADVL, and often by comparing with knockout/knockdown samples where ACADVL expression is absent or reduced. The recombinant nature ensures high batch-to-batch consistency and specificity due to defined sequence and production.

Mouse Anti-ACADVL Recombinant Antibody (V2-610911) (CBMAB-A0054-LY)

See all ACADVL antibodies

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Datasheet

SDS

Request for COA

Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

V2-610911

Application

WB, ELISA

Immunogen

ACADVL (NP_000009, 345 a.a. ~ 434 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.

Specificity

Human

Antibody Isotype

IgG2b, κ

Clonality

Monoclonal

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format

Liquid

Buffer

PBS, pH7.4

Preservative

None

Concentration

Batch dependent

Purity

> 95% Purity determined by SDS-PAGE.

Storage

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

More Infomation

Target

Full Name

acyl-Coenzyme A dehydrogenase, very long chain

Introduction

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]

Entrez Gene ID

Alternative Names

ACAD6; LCACD; VLCAD

Function

Very long-chain specific acyl-CoA dehydrogenase is one of the acyl-CoA dehydrogenases that catalyze the first step of mitochondrial fatty acid beta-oxidation, an aerobic process breaking down fatty acids into acetyl-CoA and allowing the production of energy from fats. The first step of fatty acid beta-oxidation consists in the removal of one hydrogen from C-2 and C-3 of the straight-chain fatty acyl-CoA thioester, resulting in the formation of trans-2-enoyl-CoA. Among the different mitochondrial acyl-CoA dehydrogenases, very long-chain specific acyl-CoA dehydrogenase acts specifically on acyl-CoAs with saturated 12 to 24 carbons long primary chains.

Biological Process

Energy derivation by oxidation of organic compounds
Epithelial cell differentiation
Fatty acid beta-oxidation
Fatty acid beta-oxidation using acyl-CoA dehydrogenase
IRE1-mediated unfolded protein response
Negative regulation of fatty acid biosynthetic process
Negative regulation of fatty acid oxidation
Regulation of cholesterol metabolic process
Response to cold
Temperature homeostasis

Cellular Location

Mitochondrion inner membrane
Isoform 2: Mitochondrion inner membrane

Involvement in disease

An inborn error of mitochondrial fatty acid beta-oxidation which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form characterized by early onset, high mortality and high incidence of cardiomyopathy; a milder childhood form with later onset, characterized by hypoketotic hypoglycemia, low mortality and rare cardiomyopathy; an adult form, with isolated skeletal muscle involvement, rhabdomyolysis and myoglobinuria, usually triggered by exercise or fasting.

PTM

S-nitrosylation at Cys-237 in liver improves catalytic efficiency.

Gaston, G., Gangoiti, J. A., Winn, S., Chan, B., Barshop, B. A., Harding, C. O., & Gillingham, M. B. (2020). Cardiac tissue citric acid cycle intermediates in exercised very long‐chain acyl‐CoA dehydrogenase‐deficient mice fed triheptanoin or medium‐chain triglyceride. Journal of inherited metabolic disease, 43(6), 1232-1242.

Van Calcar, S. C., Sowa, M., Rohr, F., Beazer, J., Setlock, T., Weihe, T. U., ... & Singh, R. H. (2020). Nutrition management guideline for very-long chain acyl-CoA dehydrogenase deficiency (VLCAD): an evidence-and consensus-based approach. Molecular Genetics and Metabolism.

Chen, T., Tong, F., Wu, X. Y., Zhu, L., Yi, Q. Z., Zheng, J., ... & Jiang, P. P. (2020). Novel ACADVL variants resulting in mitochondrial defects in long-chain acyl-CoA dehydrogenase deficiency. Journal of Zhejiang University-SCIENCE B, 21(11), 885-896.

Zieger, M., Keeler, A. M., Flotte, T. R., & ElMallah, M. K. (2019). AAV9 gene replacement therapy for respiratory insufficiency in very‐long chain acyl‐CoA dehydrogenase deficiency. Journal of inherited metabolic disease, 42(5), 870-877.

Yamada, K., & Taketani, T. (2019). Management and diagnosis of mitochondrial fatty acid oxidation disorders: focus on very-long-chain acyl-CoA dehydrogenase deficiency. Journal of human genetics, 64(2), 73-85.

Rovelli, V., Manzoni, F., Viau, K., Pasquali, M., & Longo, N. (2019). Clinical and biochemical outcome of patients with very long-chain acyl-CoA dehydrogenase deficiency. Molecular genetics and metabolism, 127(1), 64-73.

Watanabe, K., Yamada, K., Sameshima, K., & Yamaguchi, S. (2018). Two siblings with very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency suffered from rhabdomyolysis after l-carnitine supplementation. Molecular genetics and metabolism reports, 15, 121-123.

Hesse, J., Braun, C., Behringer, S., Matysiak, U., Spiekerkoetter, U., & Tucci, S. (2018). The diagnostic challenge in very-long chain acyl-CoA dehydrogenase deficiency (VLCADD). Journal of inherited metabolic disease, 41(6), 1169-1178.

Tucci, S. (2017). Very long‐chain acyl‐CoA dehydrogenase (VLCAD‐) deficiency–studies on treatment effects and long‐term outcomes in mouse models. Journal of Inherited Metabolic Disease: Official Journal of the Society for the Study of Inborn Errors of Metabolism, 40(3), 317-323.

Obaid, A., Nashabat, M., Alfadhel, M., Alasmari, A., Al Mutairi, F., Alswaid, A., ... & Eyaid, W. (2017). Clinical, biochemical, and molecular features in 37 saudi patients with very long chain acyl coa dehydrogenase deficiency. In JIMD Reports, Volume 40 (pp. 47-53). Springer, Berlin, Heidelberg.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
Proteogenomics
Other Protocols

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For research use only. Not intended for any clinical use.

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