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Mouse Anti-DECR1 Recombinant Antibody (3D4) (CBMAB-D0648-YC)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
3D4
Antibody Isotype
IgG2a, κ
Application
ELISA, IHC-P, WB

Basic Information

Specificity
Human
Antibody Isotype
IgG2a, 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!]

Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
2,4-dienoyl CoA reductase 1, mitochondrial
Introduction
DECR1 is an accessory enzyme which participates in the beta-oxidation and metabolism of unsaturated fatty enoyl-CoA esters.
Entrez Gene ID
1666
UniProt ID
Q16698
Alternative Names
2,4-Dienoyl-CoA Reductase 1; Short Chain Dehydrogenase/Reductase Family 18C Member 1; 2,4-Dienoyl-CoA Reductase 1, Mitochondrial; SDR18C1; DECR; Short Chain Dehydrogenase/Reductase Family 18C, Member 1; 2,4-Dienoyl-CoA Reductase, Mitochondrial;
Function
Auxiliary enzyme of beta-oxidation. It participates in the metabolism of unsaturated fatty enoyl-CoA esters having double bonds in both even- and odd-numbered positions in mitochondria. Catalyzes the NADP-dependent reduction of 2,4-dienoyl-CoA to yield trans-3-enoyl-CoA.
Biological Process
Fatty acid beta-oxidation Source: UniProtKB
Positive regulation of cold-induced thermogenesis Source: YuBioLab
Cellular Location
Mitochondrion
Involvement in disease
2,4-dienoyl-CoA reductase deficiency (DECRD):
The protein represented in this entry is involved in disease pathogenesis. A selective decrease in mitochondrial NADP(H) levels due to NADK2 mutations causes a deficiency of NADPH-dependent mitochondrial enzymes, such as DECR1 and AASS. A rare, autosomal recessive, inborn error of polyunsaturated fatty acids and lysine metabolism, resulting in mitochondrial dysfunction. Affected individuals have a severe encephalopathy with neurologic and metabolic abnormalities beginning in early infancy. Laboratory studies show increased C10:2 carnitine levels and hyperlysinemia.
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For research use only. Not intended for any clinical use.

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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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