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Mouse Anti-ACADM Recombinant Antibody (V2-179173) (CBMAB-A0430-YC)

Provided herein is a Mouse monoclonal antibody against Human Acyl-CoA Dehydrogenase Medium Chain. The antibody can be used for immunoassay techniques, such as ELISA.
See all ACADM antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
V2-179173
Antibody Isotype
IgG2b, κ
Application
ELISA

Basic Information

Immunogen
ACADM (NP_000007, 1-100 aa) partial recombinant protein with GST tag. Immunogen sequence: MAAGFGRCCR VLRSISRFHW RSQHTKANRQ REPGLGFSFE FTEQQKEFQA TARKFAREEI IPVAAEYDKT GEYPVPLIRR AWELGLMNTH IPENCGGLGL
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!]

Buffer
PBS, pH7.4
Preservative
None
Concentration
Batch dependent
Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.
Epitope
aa 1-100

Target

Full Name
acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain
Introduction
ACADM is the medium-chain specific (C4 to C12 straight chain) acyl-Coenzyme A dehydrogenase. The homotetramer enzyme catalyzes the initial step of the mitochondrial fatty acid beta-oxidation pathway. Defects in this gene cause medium-chain acyl-CoA dehydr
Entrez Gene ID
34
UniProt ID
P11310
Alternative Names
Acyl-CoA Dehydrogenase Medium Chain; Acyl-Coenzyme A Dehydrogenase, C-4 To C-12 Straight Chain; Medium-Chain Acyl-CoA Dehydrogenase; MCAD; Medium-Chain Specific Acyl-CoA Dehydrogenase, Mitochondrial; Acyl-CoA Dehydrogenase, C-4 To C-12 Straight Chain; Tes
Function
Medium-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. Electron transfer flavoprotein (ETF) is the electron acceptor that transfers electrons to the main mitochondrial respiratory chain via ETF-ubiquinone oxidoreductase (ETF dehydrogenase. Among the different mitochondrial acyl-CoA dehydrogenases, medium-chain specific acyl-CoA dehydrogenase acts specifically on acyl-CoAs with saturated 6 to 12 carbons long primary chains.
Biological Process
Carnitine biosynthetic process
Carnitine metabolic process, CoA-linked
Fatty acid beta-oxidation
Fatty acid beta-oxidation using acyl-CoA dehydrogenase
Medium-chain fatty acid catabolic process
Medium-chain fatty acid metabolic process
Regulation of metabolic process
Cellular Location
Mitochondrion matrix
Involvement in disease
An inborn error of mitochondrial fatty acid beta-oxidation which causes fasting hypoglycemia, hepatic dysfunction and encephalopathy, often resulting in death in infancy.
PTM
Acetylated. Could occur at proximity of the cofactor-binding sites and reduce the catalytic activity. Could be deacetylated by SIRT3.

Gong, Z., Liang, L., Qiu, W., Zhang, H., Ye, J., Wang, Y., ... & Han, L. (2021). Clinical, Biochemical, and Molecular Analyses of Medium-Chain Acyl-CoA Dehydrogenase Deficiency in Chinese Patients. Frontiers in Genetics, 12, 290.

Tucci, S., Wagner, C., Grünert, S. C., Matysiak, U., Weinhold, N., Klein, J., ... & Spiekerkoetter, U. (2021). Genotype and residual enzyme activity in medium‐chain acyl‐CoA dehydrogenase (MCAD) deficiency: Are predictions possible?. Journal of Inherited Metabolic Disease.

Anderson, D. R., Viau, K., Botto, L. D., Pasquali, M., & Longo, N. (2020). Clinical and biochemical outcomes of patients with medium-chain acyl-CoA dehydrogenase deficiency. Molecular genetics and metabolism, 129(1), 13-19.

Jager, E. A., Kuijpers, M. M., Bosch, A. M., Mulder, M. F., Gozalbo, E. R., Visser, G., ... & Derks, T. G. (2019). A nationwide retrospective observational study of population newborn screening for medium‐chain acyl‐CoA dehydrogenase (MCAD) deficiency in the Netherlands. Journal of inherited metabolic disease, 42(5), 890-897.

Smon, A., Groselj, U., Debeljak, M., Zerjav Tansek, M., Bertok, S., Avbelj Stefanija, M., ... & Repic Lampret, B. (2018). Medium-chain acyl-CoA dehydrogenase deficiency: Two novel ACADM mutations identified in a retrospective screening. Journal of International Medical Research, 46(4), 1339-1348.

Kaku, N., Ihara, K., Hirata, Y., Yamada, K., Lee, S., Kanemasa, H., ... & Ohga, S. (2018). Diagnostic potential of stored dried blood spots for inborn errors of metabolism: a metabolic autopsy of medium-chain acyl-CoA dehydrogenase deficiency. Journal of clinical pathology, 71(10), 885-889.

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

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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